TrueLearn Flashcards
1
Q
ABCD2 BP scores point, duration score pts, age?
A
60 min - 2 pt, 10-59 min - 1 pt
BP: >=140/90
2 pts if focal weakness
DM, age >60
DAPT if score 4, ASA only if score <4
2
Q
MS drugs MOA
A
fingolimod-binds sphingosine 1 Phosphate receptors, blocks lymphs leaving lymph nodes
- Headache
- Elevated LFTs
- Cardiac conduction abnormalities
- **Macular vol
- Abdominal distress
- Leukopenia
- Increased risk of infection
natalizumab - Ab vs alpha-4 integrin - “very late antigen 4” - blocks integrin adhesion interactions, inhibits T lymph migration into CNS
- Progressive multifocal leukoencephalopathy (PML-oligodendrocytes)
- Hepatotoxicity
- Fatigue
- Allergic reaction
glatiramer acetate - stimulates myelin basic protein (T suppressor cells)
- Injection site pain Hypersensitivity reaction, Nausea, Edema
-OK in pregnancy
dimethyl fumarate - Nrf2 pathway activation, anti inflammatory; downregulate cytokine
- Flushing
- Nausea and diarrhea
- Angioedema
- Lymphopenia
- Rare cases of PML
ocrelizmab anti CD 20
- URI
- Infusion reaction
- peripheral Edema
- Neutropenia
alemtuzumab - anti CD52 lymphocyte depletion
- Thyroid disease (Alementarium over or under eating)
- Headache
- Rash
- Abdominal distress
- Infusion reaction
- Fever
cladribine,
teriflunomide (pyrimidine synthesis inhibitor-T for thymine) -
- Teratogenic
- Hypersensitivity reactions
- Nausea
- Headache
- Hepatotoxicity
Pregnancy: can use steroids + IVIG for exacerbation
-interferons stop 1 month before conception
-glatiramer acetate ok during all of pregnancy
-interferons ok early pregnancy
3
Q
Mollaret triangle
A
inferior olivary nuc and red nuc via central tegmental tract
contralateral dentate
dentate to contralateral red nucleus via superior cerebellar peduncle;
red nucleus to inferior olivary nucleus via central tegmental tract
inferior olive to contralateral dentate via inferior cerebellar peduncle
-palatal tremor/myoclonus if ANY lesion
-can Tx benzos
-pontine infarct
Pica-Wallenberg - damage central tegmental tract (no motor weakness; ipsilateral ataxia, ipsilateral face sensation loss; no taste sense; decrease pain temp contralateral)- lateral medulla + cerebellar hemisphere (AICA at level of pons-lateral)
-SCA - cerebellar peduncle, cerebellum (no brainstem Sx)
corticate above red nucleus: disinhibit red nuc, activate rubrospinal tract -> flexor in uppers
4
Q
Hypersensitivity rxn allele carbamazepine
A
HLA-B1502
5
Q
sacral sensation
A
cauda equina - saddle anesthesia, can have normal strength and Le tone
Onuf - sphincter motor nuc S2-S4- urinary
-MSA - cause of urinary incontinence - can be presenting sign
6
Q
CIDP EMG
A
conduction block: prox CMAP amplitude decreased by greater than 50% of distal CMAP
(amplitude distal always > proximal unless supramax stim)
**temporal dispersion: >30% increase in CMAP duration **
from distal to prox stim sites b/c demyelination
-CMAP velocity <30% lower limit normal-block
prolonged distal motor latencies
PROLONGED F wave >20% normal
/ or no F wave+ CMAP amp>20% normal
-(F wave slowing out of proportion to amplitude loss)
-F waves long first change
vs amplitude decrement -> axonal
(AIDP first change ->lose F waves; H reflexes absent (stimulate tibial nerve-achilles reflex - demyelination at level of nerve ROOTs)
-check if IgA deficient before IVIG
7
Q
Martin Gruber anastomosis
A
ulnar CMAP amplitude decreased by 50% from wrist to elbow
-stimulating median N at elbow + record from ADM abductor digiti minimi - median fibers cross over to innervate ulnar muscles
8
Q
genitofemoral N, ilioinguinal N
A
genitofemoral N (L1-L2) - 2 branches:
-femoral (anterior medial thigh sense)
-genital /external spermatic N - through inguinal canal - cremaster, anterior scrotum sense
-genital N-inguinal canal with ilioinguinal N
ilioinguinal N + external spermatic N
ilioinguinal: from t12-L1 nerve roots - abdominal pain + medial thigh numbness - neuralgia during pregnancy - goes through superficial inguinal ring
(femoral N through fem triangle inferiorly)
9
Q
Tics
A
Dx- D2 R antagonists
DA agonists paradoxically help
stimulants don’ts increase tics
10
Q
kleine-Levin syndrome
A
aka recurrent hypersomnia; ideopathic
-cycles of hypersomnia + hyperphagia + hypersexuality, confusion, apathy, derealization as adolescent - 16 yrs
vs ideopathic hypersomnolence - not cyclical sleep for 11 hours, chronic daytime sleepiness, short sleep latency on MSLT
11
Q
narcolepsy type 1 vs 2
A
Need 3 months excessive daytime sleepiness
type 1- narcolepsy + cataplexy
-low hypocretin-1 in CSF OR cataplexy
-sleep hallucinations
-HLA-DQB1*0602 gene
-loss orexin= hypocretin from lateral hypothalamus
type 2 - no cataplexy, normal hypocretin-1 in CSF
Dx: polysomnigram + multiple sleep latency test
12
Q
rabies
A
-encephalitis, hydrophobia, seizures, N, V, agitation
-temporal lobes, limbic system
Negri body: dark oval large spot around perimetry of cell; can be ultiple
13
Q
Herpes encephalitis
A
Cowdrey A
-intranuclear, solitary, surrounding halo - large pink blob
14
Q
Hirano body
A
Alhzheimer’s
-aggregate actin proteins
15
Q
EPS
A
from D2 R block
acute dystonia - IV diphenhydramine - 5 days drug exposure
Torticollis - Tx Botox (torticollis rotate to side vs laterocollis - head tilt)
Akathisia - inner FEELING-most common D2 EPS
switch to low potency - quetiapine
tardive dyskinesia - 3 months neuroleptic use - chorea = brief, unpredictable abrupt irregular movements
slow writhing - athetosis
slow twisting movements - tardive dystoni
16
Q
NREM parasomnia
A
Night terrors - arousal, screaming, crying + tachy, diaphoretic; confused when woken up
vs nightmares - patients not confused
vs Panayiotopoulos syndrome - btw 3-6 yrs; +N, +V
17
Q
ideamotor apraxia
A
localizes left hemisphere
18
Q
back pain/radiculopathy w/u
A
Imaging not until 6 weeks of Sx
diagnosis: spinal imaging after 6 wks
EMG - confirms Dx
if C6 radic b/c can present like carpal tunnel so need if C6
19
Q
Mitochondrial disease
A
Kearns Sayre
ragged red fibers on biopsy
-retinitis pigmentosa
-progressive external ophthalmoplegia
-DELETIONS of mitochondrial DNA or rearrangement
Leber hereditary optic neuropathy - point mutations mitochondrial dna
myoclonic epilepsy with RRF - common
Melas - very rare - transfer RNA point mutation
20
Q
filum terminale causa equina conus medullaris
A
Conus medullaris tapered end of SC then cauda equina nerves
Filum terminale-sack of pia mater, threadlike structure at end of conus medullaris that anchors to cococyx
21
Q
Mydriasis: Adie’s pupil + CNIII
A
Adie: no light reaction, intact near reaction - light-near dissociation
Mydriasis, tonic pupil, b/c parasympathetic denervation; (chronic)
sluggishly react to light, can accommodate with near response
-
-test: PILOCARPINE -cholinergic - Adie pupil constricts a lot b/c of denevation hypersensitivity
-normal pupil should not constrict with dilute pilocarpine
-little old Adie: pupil small with time
Idiopathic or ciliary ganglion lesions
-react poorly to light but
-after viral infection
CN III palsy - accommodation affected equally; affects E-W fibers in parasympathetics
-CN III palsy: concentrated pilocarpine: constricts CN III palsy
22
Q
pallister hall syndrome
A
hypothalamic harmartomas
GLI3 gene
-gelastic seizuers - preserved awareness - last 30 sec -refractory to ASMs
23
Q
Billing medicare/Medicaid
A
No level 1
2 - <30 min new; <20 min old
3-<45 min new; <30 min old
4-<1 hour new; <40 min old
24
Q
Leukodystrophy
A
CADASIL - NOTCH3 AD - subcortical white matter - strokes, dementia
Alexander - GFAP Chr 17 AD-rosenthal fiber deposits in astrocytic processes - involves U fibers, macrocephaly - BG + thalamus + frontoparietal atrophy
CAnAvAn – aspartoacylase ASPA AR - spongy degeneration includes spinal core involves U fibers, macrocephalic subcortical
Krabbe - AR galactocerebrosidase GALC Chr 14- globous cells (child/adult/adolescent form)-tripnormal early; later atrophy thalamus, splenium, brain stem, IC; vs metachromatic leukodystrophy
Metachromatic leukodystrophy - arylsulfatase A AR- ARSA - spares U fibers, thalamus, splenium, IC; Tigroid pattern, periventricular
Vanishing white matter: eIF-2B-related disorder - translation protein mutation - stressor like illness then hypotonia, ataxia, motor dysfunction - MRI: white matter radiating strips
X L adreno leukodystrophy - Posterior changes on MRI
25
Tx spasticity
-oral meds then intrathecal baclofen - generalized spasticity
GABA B R agonist - baclofen
(Etoh Gaba A)
-risk pump malfunction -> withdrawal/overdose
-withdrawal: seizures, hyperthermia, DIC, rhabdo
-Botox injections
-PT if only mild spasms
refractory - rhizotomy - ablation dorsal rami nerves
(tizanidine - alpha 2 agonist - presynaptic motor nerves; lower BP)
26
Guyon canal vs cubital tunnel
Cubital tunnel - elbow - wrist flexion + addiction weakness
Guyon canal - at wrist -
27
Palliative care
Dying discussion limited by physician not patient or family
-optimal to start palliative care at the time of life threatening or life limited illness
28
Fregoli vs cotard delusion vs pseudocyesis
Fregoli - believe everyone is actually 1 person in various disguises
Cotard - believe they are dead/dying
Pseudocyesis - believe you are pregnant
29
Benzo wean
Reduce by 25% every 2 weeks
30
Posterior cortical atrophy
Alzheimer variant with visual impairment, simultanagnosia, oculomotor apraxia
31
Pick body vs Lewy body
Pick: off center black spot
Lewy body: round spot +/- halo; in old person
-lewy body dementia - quetiapine ok (pimozide typical antipsychotic, don't use)
32
Meiosis: Horner + Argyll-Robertson
Argyll-R: small pupils don’t react to light,+ react to accommodation
Horner: Dx - interruption sympathetic pathways from hypothalamus
-cocaine drops; inhibit NE reuptake, more NE= Mydriasis (in Horner’s, no Mydriasis; anisicoria more prominent b/c normal pupil larger)
-apraclonidine: Horner pupil larger b/c NE R activated, hypersensitivity in Horner’s causes pupil dilation (normal pupil doesn’t change)
Hydroxyamphetamine: preganglionic lesion dilates, post ganglionic lesion stays same
33
Aminoacidopathy
Nonketotic hyperglycinemia- GLDC, AMT; AR
Early myoclonic encephalopathy
Elevated glycine, NO ketones (vs organic acidemias)
34
Imaging pregnant vs postpartum
No iodine contrast or gad during pregnancy; can use gad if you have to; gad and iodine is ok for breastfeeding
Pregnant: no contrast
Papilledema- get MRV without (MRV+ if post partum even if breastfeeding)
Preeclampsia: get MRI without
PRES: MRI without if pregnant; with contrast post partum
with contemporary CT imaging, attributable risk of cancer to the radiation exposure low
gad ok in breastfeeding
35
Tetrabenazine
VMAT inhibitor
Chorea in Huntington
Vs trihexyphenidyl- anticholinergic - Tx EPS side effects
36
Meige
Botox ONLY; not carbamazepine
37
Gelastic seizures
Refractory seizures lasting 30 sec
Pallister-Hall -GLI3 - hypothalamic hamartoma
38
Benign myoclonus early infancy
Before 1 yr, spasms self limited; awake + alert
39
Galantamine
AChe antagonist+ allosteric nicotinic modulator
Side effects GI
40
Respiratory patterns
Cheyne stokes - BL hemisphere, thalamus
-oscillate btw hyperventilation + hypoventilation
crescendo/decrescendo then apnea, see in sleep
Deep sighing breaths - Kussmaul - DKA
Irregular - ataxia - medulla
Irregular+ shallow - agonal - diffuse anoxic injury
Midbrain (ventral): Central neurogenic hyperventilation - Regular breaths >40 RR , continues during sleep-CNA lymphoma-medial pons
Pons tegmentum - apneustic - prolonged inspiration with 2-3 pause then expiration - SAME APNEAS
Lower Pons (dorsolateral) - cluster breathing - breaths with similar depths then VARIABLE APNEAS
-can see with MSA
Medulla - ataxic - dorsomedial - IRREGULAR in rate, rhythm, amplitude
Lateral medulla, high cervical cord - central neurogenic hypoventilation - loss of respiration during sleep
41
Vert dissection trauma vs spontaneous vs vert A stenosis
Trauma: V3- C1-C2
Spontaneous dissection: V4 - Wallenberg syndrome
Vert A stenosis - V1
42
storage diseases
Fabry - can present in adulthood - renal - amyloid deposition - A Gal who tries ceramics
alpha galactosidase , accumulate trihexyceramide
-pottery wheel splatter: heart + renal (lipid deposits) + angiokeratomas + small fiber neuropathy (hands + foot pedal) +strokes/CV disease (clay clogs)
histo; lipid accumulation in blood vessels
vs gaucher, krabbe - less likely to present as adult
Niemann Pick - foam cells
-A - severe, B; later onset - SMPD1 - sphingomyelin phophosdiesterase-1
type C - NPC1/2 mutations - cholesterol transport
43
neurocognitive testing - learning disability
**isolated low memory score** - Visual memory
-Stroop - disinhibition, executive fxn
finger tapping, symbol digit coding- psychomotor speed
(abnormal in concussion)
44
GPi vs DRG vs putamen damage toxicity and treatment
GPi - CO, manganese
putamen - methanol + optic nerve
DRG-B6 excess
methanol - Tx ethanol
ethylene glycol - Tx fomepizole in kids, ethanol in adults
45
trinucleotide repeat expansion
CAG - spinocerebellar ataxia (SCA1-ATXN1 and on other genes); Huntington (Caudate has low ACh + GABA) - need 40 repeats for full penetrance
-spinocerebellar ataxia vs Friedreich - can be older, increase DTRs, no sensory loss
GAA-Friedrich’s ataxia - GAAit ataxic (present 5-15, low DTRs but + babinski, sensory loss)
CGG-fragile X - chin (protrudes), giant gonands
CTG-myotonic dystrophy - cataracts, toupee, gonadal atrophy
46
vascular malformations
dural av fistulas - don’t see on MRI -see on angio
47
patient-physician relations; transition ped to adult care
primary non adherence - medication never filled - non fulfillment adherence
secondary non adherence
-non-persistence - start med then stop
-non-conforming - taking med not as prescribed
Patient physician discordance - non adherence - misunderstand doc
Transition to adult care: Transition Readiness Assessment Questionnaire- can bill for; start at age 14; confidential to parents
48
cerebral contusion
-chronic hypodensity - looks like black cystic space
-contre-coup injury
49
chiari ii vs dandy walker
**Chiari 0: syringomyelia with mild hernation <3 mm** - NORMAL VARIANT
- chiari I - progressive suboccipital HA-syrinx: can resolve spontaneous with surgical fix in
-tonisal herniation >5 mm inferior to foramen magnum
-size of posterior cranial vault prediction of Sx severity, NOT degree of tonsillar herniation
-McRae line - line at base on foramen magnum - Chari crosses
chiari II: hydrocephalus + **myelomeningocele**
dandy walker: agenesis cerebellar vermis
iii, iv - no myelomeningocele, only II
chiari iii: cerebellar herniation into encephalocele +/- brainstem
chiari IV: cerebellar hypoplasia/aplasia with normal posterior fossa
50
split cord 1 vs 2
split cord 1: 2 hemicords with own dural sheath, separated by septum
split cord 2: 2 hemicords in 1 dural sheath
sx: tethered cord, gait disturbance, atrophy LE, spasticity
51
currarino triad
anterior sacral meningocele + presacral mass + anorectal abnormality
52
MEG
focal epilepsy on EEG + no structural lesion on MRI
53
lipomyelomeningocele
cutaneous stigmata: hypertrichosis, hyperpigmentation, palpable lipoma
54
Bunina bodies vs hirano bodies
ALS - cytoplasm motor neurons
Hirano: rod shaped, pyramidal CA1 hippocampus
-normal aging or EtOH, Alzheimer’s, Pick’s
55
breath holding spell; shuddering attack
supplement with iron reduces attacks
reproduce: 10 second ocular pressure during EKG->bradycardia/asystole
-incontinence, clonus then sleepy
-no CTH emergent unless prolonged LOC ; can wait for MRI
shuddering attacks: rapid bursts of trembling, benign, normal EEG with myogenic artifact 20-100 Hz
-occur when excited
56
Parkinson’s management
Half life L dopa: 1.5 hours
L-dopa incude dyskinesias Tx:
-**amantadine** - risk hallucinations - NMDA antagonist
(Sx-constantly needing to move)
-mottled rash - STING RAY
-increase frequency decrease dose L dopa/continuous infusion; continuous apomorphine subcu infusion
-DBS- <75 yrs, **clear response to L Dopa **but dyskinesias or off state; cognitive intact, psych intact
-only partially affective for tremor
-no dementia
-GPi, STN (NOT VIM - for essential tremor)
urinary urgency - Tx mirabegron - beta 3 agonist (less cholinergic vs oxybutynin)
Hallucinations - (infectious w/u) first withdrawal anticholinergic then amantadine then DA agonist
PD psychosis -
Tx - pimavanserin. **5-HT2A R agonist**
REM sleep behavior predates LBD + PD
+/- MSA
MRI: ABSENT swallow tail sign = loss of pigmentation in the substantia nigra
PD dementia: RIVASTIGMINE approved
57
PD drugs
pramipexole - DA agonist
Rasagiline - MAO-B inhibitor
Entacapone - COMT
amantadine - NMDA antagonist
-also hastens recovery in comatose pts 16-65 when given 4-16 wks pos-tinjury
L-Dopa side effects - GI upset, nightmares, hallucinations, dizziness
58
PTSD length Sx, kids; cyclothymic, panic disorder
1 month
Sx different in kids vs adolescents - can have disorganized/agitated behavior, not fear
-physical proximity to trauma doesn’t matter
cyclothymic: TWO YEARS Sx
panic disorder: 1 month and one attack, Tx= desensitization and exposure therapy
59
NCSE vs postictal
NCSE - positive symptoms: mydriasis, hiccups, nystagmus, eye deviation
60
sleep apnea
apneas: no respiratory for 10 sec; hypopnea = reduction air flow 30%
Apnea/Hypopnea index per hr sleep
mild 5-14
moderate>15
severe>30
central: pontomedullary respiratory center problem
absence airflow+**abdominal + thoracic movements** - on polysomnogram
-heart failure (cheynes stokes)-> get ECHO
vs. obstructive - closed airway
-OSA; incidence the same in post menopausal women and men
61
sandy orange diapers, UTIs
Lesch Nyhan
HPRT mutation
62
dermal sinus tract
terminate in SAH, bone, etc - meningitis
63
split cord 1 vs 2
diastematomyelia
1 - 2 cords 2 sacs
2 - 2 cords 1 sac
64
curriano triad
anterior sacral meningocele + sacral mass + anorectal
-more common in women -urinary Sx
65
spina bifida occulta vs myelomeningocele
spina bifida occulta - folate deficiency - no vertebral body fusion (vs rickets - not vertebral column defect), see lucency in spine
-Depakote (neural tube defects)
myelomeningocele - chiari ii + hydrocephalus
66
Isaac syndrome
Autoimmune disorder - paraneoplastic ; -voltage gated potassium channelopathy
Sx: carpopedal spasm, sweating, myotonia
peripheral nerve hyperexcitability -
-EMG: neuromyotonia ( ping sound , high frequency 150-300 Hz decrementing repetitive discharges) + myokymia - bag of worms continuous muscle twitching
vs. myotonia - 20-100 hz - waxing/waning dive bomber sound
67
EMG: fibrillation potentials vs complex repetitive discharges vs positive sharp waves
denervation: fibrillation physiologic equivalent of positive sharp waves
-Fibrillation - negative component first then positive - denervation (denervation goes down)-“rain on roof” sound
-positive sharp waves - downward deflection waves - denervation
complex repetitive discharge - perfectly repetitive spikes machine like sound
-depolarization of single motor unit spreading to adjacent fibers; high frequency 5-100 Hz
end plate spikes - seashell sound on EMG, low amplitude potentials- up component first then down-normal on needle insertion
68
Postural orthostatic tachycardia vs neurogenic orthostatic hypotension vs vasovagal
POTS-head up increase HR by 30 or more over baseline or >120 per minute
-HR the same
Dx with tilt table
-tx: aerobic exercise, hydration, steroid, midodrine (**alpha-1 agonist**; causes urinary retention), beta blocker
neurogenic orthostatic hypotension= SBP drops 20 or DBP drops 10 when standing
= MSA, autoParkinson’s (and no increase HR)
Reflex syncope -includes vasovagal defecation/micturition/coughing/swallowing - need EKG
neurocardiogenic=vasovagel
-heart causes exaggerated sympathetic response and brainstem withdrawals sympathetics causing vasodilation
orthostatic hypotension response - general visceral efferent
95% specific to seizure: lateral tongue bite
69
Phenytoin
zero order kinetics = within therapeutic range, PHT half life increases and serum concentrations increase nonlinearly proportional to dose
-nystagmus, ataxia, confusion
-purple glove syndrome - after load, blistering skin
toxocity: gingival hypertrophy, ataxia, diplopia, slurred speech
70
Zonisamide
weak CA inhibitor -> metabolic acidosis
sulfonamide related
blocks T type calcium channels
-can get kidney stones
71
lamotrigine
OCP decrease lamotrigine-estrogen-progestin
-lamotrigine reduces effectiveness of ocps
lamotrigine + VPA = increase SJS risk
-VPA inhibits UGT-glucuronidation->raises LTG
72
poinsoning
arsenic - AIDP, sensory motor neuropathy, garlic breath (GI Sx acutely)
moonshine, pesticides, mining, glass
manganese - parkinsonism - HA, psychosis
GPi hyperintensity (+ CO)
-in liver disease pts, parkinsonism, wedling
Tx: chelation
cyanide - almond, HA, anxiety, vertigo, seizures, encephalopathy, skin flushed
-mining, electroplating, domestic fires fires
mercury - psychosis, gum inflammation, ataxia
organophosphates - cholingergic tox
-miosis vs botulism
73
tetanus vs tetany
toxin decrease interneuron transmission
-disrupts fusion synaptic vesicles
clostridium tetani-toxin targets presynaptic synaptobrevin in spinal anterior horn-> no glycine release->no inhibitory interneurons ->spasm
*ANS dysfxn*
EMG: high frequency discharges after single stimulus
vs. toxin completing with glycine at postsyn motor neuron - strychnine - drugs, herbal meds
TETANY: acute hypocalcemia - high frequency discharge after stimulus
(high frequency rep stim for botulism)
74
conduction velocity dependence factors + pacemaker
Low/slow CV: low temp, low diameter, low myelination, **decreased internode length, tall person**
High/fast CV: larger axon, warm, more myelinated, **long internode length=faster**, short person
Saltatory conduction: myelinated fibers CV 35-75 m/s vs (unmyelinated 0.2-1.5 m/s)
*myelin decreases capacitance*=less current lost
sodium channel density highest at nodes
pacemaker: can use but limit number electrodes and do not stimulate near device; stimulus shorter than 0.2 and frequency 1Hz
75
Spinal lesion
**ependymoma - most common SC tumor**- 40 years - intradural intramedullary - CERVICAL-central-bilateral arm weakness and loss of pain + temp
Lymphoma SC - intramedullary - CERVICAL mets
vs syringomyelia - CERVICOTHORACIC, signal in cord - HORNER syndrome b/c descending autonomics
-spares dorsal columns
-cervical spondylosis/**hyperextension injuries, syringomyelia**, intramedullary tumor-> central cord syndrome
meningioma - THORACIC spine-peak in females 70-80 yrs (in the MIDDLE)
epidural -hematoma - spontaneous in kids - THORACIC spine - T2 hyperintense, T1 iso-hypointense in epidural space, T2 bright in cord
-evacuate in 6 hours= better outcome
Thoracic radiculopathy - truncal radicular pain - diabetes risk
-DDx shingles
vs epidural abscess - LUMBAR, abscess, edema
myxopapillary ependymoma - 30s, M>F, LUMBAR
Neuroectodermal tumors - FILUM terminale, CAUDA equina - heterogenous enhancing - aggressive
vs transverse myelitis - edema; one spinal level
vs astrocytoma - intramedullary lesion, *edema*
spinal cord hemorrhage - hypointense on GRE, heterogenous signal on T1/T2 (vascular malformation) vs. compressive lesion - bright T2, dark T1
76
cryptococcus source
birds - pigeon poop on mummies
77
tetany
Acute hypocalcemia - high frequency discharge after single stimulus
-chovstek (contraction facial mulcles when tap facial nerve); trousseau - BP cuff inflate -> carpopedal spasm (caused by ischemia)
78
botulism vs MG vs single fiber
botulism: toxin inhibits presynaptic ACh release via cleave SNARE; autonomic dyxfsn
-ACh Presynaptic for the SNS + PNS system; (ACh post synaptic for PNS) = **pupillary dilation and low DTRs**
- **NC: low CMAP with facilitation after exercise or HIGH frequency 20-50 Hz rep stim** (like LEMS); jitter abnormal
-fermented seafood
-neonatal MG: intact DTRs
**MG: EMG: low frequency stimulation 2-3 Hz**, decrease response on rep stim
-no pupillary dilation vs botulism
-MG: post synaptic inhibition (Botulism pre synaptic inhibition)
vs tetanus - continuous motor activity
Single fiber: Jitter - variation in time interval btw 2 AP
-97% sensitivity for ocular MG
99% sensitivity for generalized MG
ACH R Ab: 50% sensitivity ocular MG, 85% sensitivity generalized MG (if seronegative, MUSK positive in 50%)
79
Hyperhidrosis
Primary Hyperhidrosis - ideopathic - axillae palms soles, face, scalp, inguina, etc; bilateral and symmatric
-get skin infections
+sweating during day only
+ family history
Dx: starch iodine test (different powder than thermoregulatory)
Tx: glycopyrrolate - oral -side effects *vomiting, constipation*
-aluminum salts - antiperspirants
Botox
Endoscopic thoracic sympathectomy - destroys sympathetic ganglia
Regional hyperhidrosis -bilateral axilla, feet, palms, face
secondary hyperhidrosis - night sweats- awake and asleep
syringomyelia - episodic sweating with some posture, autonomic dysreflexia
80
autonomic testing
Test **Sympathetic Sudomotor cholinergic**
sweat testing-
thermoregulatory testing - POWDER - changes color, shows pattern where someone sweats -
-ex autonomic neuropathy
Quantitative sudomotor axon reflex testing - stimulate nerves innervating sweat glands-postganglionic
**Sympathetic cardiovascular adrenergic -*HR, BP with Valsalva, tilt table**
**HR response to deep breathing - PNS cardiovagal** /Valsalva (impaired in diphtheria)
81
vitamin e deficiency
can happen with UC and crohn’s
82
mononeuritis multiplex
asymmetric stepwise progression cranial or peripheral neuropathy
VASCULITIC + systemic symptoms, weight loss
**NC/EMG: axonal neuropathy**, no demyelination. can biopsy
-vasculitis, autoimmune disease
83
spinal muscular atrophy
SMN1 - AR -mRNA processing gene - (Chr 5)
-loss of SMN1 compensated by SMN2 (more SMN2 the better)
- abdominen protrusion b/c diaphragm weak
-minipolymyoclonus - fine tremor of limbs
-spares facial muscles+ has fasciculations vs congenital myasthenia (ACh R delta subunit mutation) - ptosis, choking, Sx worse end of day
neonatal MG - maternal ACh R Ab transmitted to fetus - hypotonia, **intact DTRs**
SMA0: prenatal - no motor milestones, resp failure by 1 month - 1 copy SMN2
**SMA1: unable to sit (Werdnig-Hoffman) - onset before 6 months
1-2 copies SMN2**
**II: unable to walk - onset after 6 months-3 copies SMN2**
III: able to walk - onset>18 months- 3-4 copies SMN2
EMG-complex repetitive discharge
-SMA4-adult - proximal muscle weakness esp quads - more than 4 copies SMN2
Tx: nusinersin
onasemnogene abeparvovec - SMN1 transgene vector Tx <24 months
84
sympathomimetics
VMAT inhibitor: deplete DA in presyn vesicles -reserpine - tardive
cocaine: inhibits presynpatic DA and NE reuptake - NET DAT CAT - cuts net of fish
amphetamine: presynaptic RELEASE NE, 5HT, DA
(LSD - 5-HT)
-amphetamine toxicity - fluids, benzos, acitvated charcoal
85
chronic traumatic encephalopathy
TAUopathy - hyperphosphorylated tau widspread
-1 perivascular phosphorylated Tau lesion around blood vessel - in sulci of cortex
-multiple concussions -> apathy, aggression, personality change
**cerebellar scarring, substantia nigra**
-degeneration, atrophy, cavum septum pellucidum —>>> temporal lobe, thalamus, hypothalamus, mammillary body atrophy
vs. Prion disease - spongiform degeneration - no inflammatory response, DWI cortical ribbon
86
Carnitine deficiency disorders -Lipid metabolism beta oxidation
Carnitine palmitoyltransferase II deficiency-CPTII gene
- myopathic form: most common - pain and weakness **high CK + myoglobinuria after exercise/stress only**
-MCC hereditary rhabdomyolysis
-other types neonatal, severe infantile hepatocardiomuscular
-trigger: diazepam, **VPA**- high ammonia
EMG-normal; biopsy normal
-Dx: elevated long chain acylcarnitines, low free canitine
Tx: carbs, low fat
-CPT1A deficiency - fatty liver pregnancy if carrying infant with CPT1A - carnitine HIGH in serum
carnitine acylcarnitine translocase deficiency - SLC25A20 - low free carnitine
-carnitine biosynthesis disorders
-carnitine transport disorders - primary carnitine deficiency (SLC22A5)
dialysis related carnitine disorder - fatigue, hypotension, anemia - free carnitine cleared more than acyl carnitine in dialysis
secondary carnitine deficiency - dialysis, drugs, inborn errors metabolism (increase excretion acylcarnitines), malnutrition
- less severe vs primary deficiency
87
Glycogen storage diseases
-McArdles myophosphorylase - *SECOND WIND* clenched fist, difficulty opening hand; contractures, rhabdomyolysis
-2nd wind b/c free fatty acids delivered
Tx: carbs
Lysosomal acid alpha-glucosidase - acid maltase deficiency - Pompe
-adults: proximal muscle weakness, atrophy
Tx - alglucosidase alfa
Glucose-6-phosphatase deficiency - Von Gierke - hepatomegaly, hypoglycemia, growth retardation; high lactate, uric acid
Galactose-1-P-uridyltransferase - galactosemia - cataracts, failure to thrive, aminoaciduria
liver/muscle debranching - Cori - hypoglycemia, cardiomegaly, hepatomegaly
88
transient global amnesia
can be triggered by coitus or emotional/physical stress
89
Parkinson’s pathology and genes
eosinophilic cytoplasmic inclusions = Lewy bodies - pale halo
-dopaminergic denervation striatum
-pallor substantia nigra
-pallor locus coeruleus
Late onset; LRRK2
early onset - DJ1 - AR, PINK1 - AR
PARK1 - AD
PARK2 - AR
90
neurotransmitters basal forebrain, histamine
basal forebrain - ACh,
substantia innominata
nuc accumbens
91
myoclonus Tx
Keppra, VPA, benzos (clonazepam, midazolam), topamax
worsen myoclonus: carbamazepine, phenytoin, gabapentin, lamotrigine
92
Sydenham chorea
Tx - penicillin + DA antagonist
-fluphenazine
93
restless leg syndrome
augmentation: DA meds cause Sx early in day, more severe, other body parts (can happen after 1 month)
kids - check iron panel, can have comorbid ADHD, periodic limb movements of sleep
Tx iron deficiency ; **first line gabapentin/pregabalin**
**Tx OSA improves Sx**
94
Histopathology
Target fibers - succinate dehydrogenase staining - denervation
Cowdry A inclusions - herpes
Plasmodium - hemozoin - malarial pigment
toxoplasmosis - bradyzoites
schwanommas - Antoni A + B cells
metastatic adenocarcinoma - simple and tubular glandular cells, rim enhancing cystic mass
95
factitious vs malingering
factitious - want to be a patient , no secondary gain
malingering - needs secondary gain like money or disability or drugs
96
RNS DBS treatments
RNS: multifocal or focal and not a surgical candidate
-gives stimulus at seizure onset, closed loop system (vs DBS, VNS - open loop)
-side effects less common in RNS vs. VNS+DBS b/c give focal stimulus
DBS: 1st- approved for essential tremor - Ventral intermediate nuc thalamus
2nd- dystonia - GPi
3rd OCD
4th focal onset epilepsy - ant thalamus - 2008
5th - MDD treatment resistant - most recent
97
Brain tumors kids
INFRATENTORIAL
Pilocytic astrocytoma - Cerebellar Hemispheres (medulloblastoma is midline and roof of 4th ventricle
-cyst with enhancing mural nodule
-if NF1 -> in optic tract
-presenting Sx MASS EFFECT
-bipolar neoplastic cells with hair like processes (Rosenthal fibers)+eosinophilic granular bodies (eosinophilic hyaline masses)
-fusion K-BRAF protein
Tx: surgery, BRAF inhibitor
Ependymoma - 4th ventricle FLOOR-perivascular pseudo rosettes
Medulloblastoma - MIDLINE vermis or ROOF fourth ventricle - Homer Wright Rosettes
(DROP things from the ROOF)
-age 2-6
4 subgroups: WNT, SHH, group 3, group 4
1. WNT - BEST prognosis - CTNNB1 mutation (kids, adults)
2. SHH - PTCH1 gene, MYCN, GLI2 - intermediate - infants, adults
3. **group 3- worst prognosis - MYC amplification** - infants, children
4. group 4- CKD6 (MYCN)- intermediate prognosis
-Homer-Wright pseudorosettes
-get spinal imaging b/c drop metastasis
-Turcot syndrome - gliomas, colon polyls, adenocarcinoma, medulloblastoma - APC
SEGA - 3rd or lateral ventricles
-TSC only
Histo: -calcifications
-candle gutterings
SUPRATENTORIAL
Pleomorphic Xanthoastrocytoma - temporal lobes
-histo: intercellular *reticulin* deposit
Ganglioma - temporal lobe
hemangiopericytomas - staghorn vasculature
98
Supratentorial tumors
SATCHMOE - sarcoid, aneyrusm/adenoma, teratoma, craniopharyngioma, hypothalamic glioma, mets, opic nerve glioma, epidermoid
1. Ganglioglioma - temporal - cyst with mural nodule - BRAFV600E
-neuronal + glial on histo
2. Embryonal tumor with multilayered rosettes - pseudostratified epithelium with central clear lumen - heterogenous contrast enhancement, **diffusion restriction**
-frontal, parietotemporal
-ages <5
-C19MC amplification on chromosome 19
if no C19M classification but has pseudostratefied neuroepithelium -> medulloepithelioma
Dysembryoplastic neuroepithelial tumor - DNET - no contrast enhancement or diffusion restriction - glioneurla nodules, foci cortical dysplasia
Pleomorphic Xanthroastrocytoma
Craniopharyngioma - Rathke pouch - palisading epithelial cells; cystic component
-calcifications
-papillary craniopharyngioma - non keratinizing squamous epithelium + fibrovascular core
vs pituitary adenoma - hypercellular with same cell type
99
cyst with mural nodule DDx
pleomorphic xanthroastrocytoma
ganglioma
pilocytic astrocytoma
hemangioblastoma - cyst usually does not enhance
100
radiation plexopathy
MRI - enhancement - tram track appearance
FDG-PET - no uptake vs tumor
EMG-myokymia (worm like movements under skin) - sound like soldiers marching + fasiculations
not painful vs. tumor infiltrative plexus
101
confusional migraine
Rare Pediatric migraine
speech slurred, vomited, disoriented, agitated x 6-24 hours
Tx. topiramate (blocks VG NA and Ca channels + blocks CA, enhances Gaba
102
Moya moya
vasculopathy of narrowed distal ICA + branches
watershed strokes
puff of smoke
-limb shaking, chorea - disrupt BG circuit
-**no inflammation or athero on biopsy** -> not inflammatory
-intimal hyperplasia, interruption internal elastic lamina, proliferation sm muscle
Tx - ASA, no AC
103
craniosynostosis
one sutures or more closes early
**primary - ossification problem
if 2 sutures close early -> increased ICP**
-sattigal suture - scaphocephaly; coronal - anterior plagiocephaly - bilateral coronal - brachycephaly
lambdoid - posterior plagiocephaly; metopic - trigonocephaly
-Crouzon - fibroblast growth factor-2 gene. bicoronal craniosynostosis - brachicephaly, proptosis, midface bony hypoplasia - hands normal vs Aperts, Pfieffer
secondary -most common- small brain growth-> early fusion -**MICROcephaly**-
104
NMO, ADEM
BL optic neuritis
aquaporin 4 channel - astrocytic foot processes, gray matter spinal cord
acute Tx - IVIG, steroids, PLEX
Interferon Beta can worsen disease b/c -B cell mediated disease (INF B T cell)
natalizumab, fingolimod worsens disease
reduce relapse - rituximab, azathioprine, eculizumab
For:
**ADAM Tx - steroids 1st line**, then plasma exchange, IVIG
105
Cruciate paralysis
cervicomedullary junction
brachial diplegia -
**bilateral arm weakness, spares legs** **SPARES SENSation + dysarthria/dysphagia (lower CN)**
-CST with arms decussates one segment higher than legs, more anterior, more susceptible to injury
-vs. C spine - arms and legs and sensation affected
-odontoid fracture, atlantoaxial
106
B12 deficiency
no Methylmanonyl CoA to succinyl coA-> high MMA
107
dopamine precursors
phenylalanine, tyrosine
108
infantile spasm
ACTH first line
genes: STXBP1
vigabatrin if TSC - causes VF deficits
-inhibits GABA-T -> increases GABA
hypsarrythmia - best seen during quiet sleep - need 24 hrs EEG
109
ethosuxamide side effects
N, V, sleep problems, hyperactivity
110
Migraine neurostimulation + biofeedback
Vagal nerve stimulation - **cluster** + migraine
DBS not approved for cluster
migraine abortive + preventative: transcranial magnet-nTMS ,external trigeminal stimulation; vagal nerve stim, occipital stim
Cefaly - transcutaneous supraorbital neurostimulation - episodic migraine - forehead device - use once/day x 20 min - stimulate supraorbital N (trigeminal N branch)
Biofeedback: migraine prevention - control physical response to stress- body functions not under conscious control - (not Emotional responses)
111
essential vs physiologic tremor
physiologic = able to perform tasks
-symmetric
-risk with SSRI
essential tremor - asymmetric, worse with action
-don’t need to check serum copper
-postural tremor - worse in wing beating vs arms out
112
concussion
prognostic indicator: more severe Sx within first few days
-severity of concussion does not correlate with post concussive syndrome risk
113
Dx bipolar I
Manic episodes lasts 7 days
-increase in goal directed activities
-don’t need depression in bipolar I
(bipolar II-hypomanic + MDD)
114
cervical myelopathy
115
ADHD
if >6 years: stimulants ; no increase dependence
if epilepsy - low dose methylphenidate
-if tic disorder/ASD aggress - alpha 2 agonists - clonidine - better if comorbidity
116
Perilymphatic fistuala
perilymph fluid leads from inner to middle ear
-vertigo, disequilibrium
-sensorineurla hearing loss, left beating nystagmus
Tulio phenomenon: disequilibrium triggered by loud noise or valsalva
cause: congenital abnormality temporal bone or trauma
vs Meniere - dilation endolymph - Sx better with salt restriction; diuresis to decrease endolymph
Tx - **endolymphatic hydrops**
117
refractory epilepsy neonates
pyridoxine dependent epilepsy
-deficiency alpha-aminoadipic semialdehyde dehydrogenase - antiquitin
-clonic seizures
118
Phantom limb pain vs residual limb pain
phantom limb: more common upper limb
peripheral + CNS hypersensitization
vs residual limb pain: associated with underlying cause
119
CVST w/u
CTH THEN MRI MRV before LP
-get bHCG
120
Foster Kennedy Syndrome
Mass compressing optic nerve, ipsilateral optic pallor/atrophy, contralateral papilledema, anosmia
ex: sphenoid wing meningioma
121
Benign idiopathic neonatal convulsions and benign familial neonatal seizures vs benign familial infantile seizures
1. Benign idiopathic neonatal convulsion
fifth day fits, seizure 4-6 days
-variant theta rhythm on EEG
-clonic seizures
-KCNQ2 - deletion (loss of function of 1 gene = 50% function)
Vs mis sense mutation KCNQ2 - gets incorporated into channel and even less function (less than 50%) - get DEE not fifth day fits
2. Benign familial neonatal seizures
KCNQ2, KCNQ3 gene, AUTOSOMAL DOMINANT
-EEG NORMAL, MRI normal, normal growth/dvelopment
Tx: no tx or oxcarb, carbamazepine
3. benign familial infantile seizures; SCAN2A, SCAN8A - Autosomal dominant
-start at 6 months and seizures end at 2 years
-association with adult paroxysmal kinesigenic dyskinesia in adolescence-PRRT2 gene - proline rich transmembrane protein 2 gene - Chr 16 p
febrile seizures-GEFS+. SCN1A
122
Hypothalamus
lateral - orexin
suprachiasmatic - insomnia, sleep wake
arcuate - > secretes stimulating and inhibitory releasing hormones - tuberohypophyseal tract
paraventricular nucleus - autonomic fibers
anterior hypothalamus - regulates hyperthermia
lesion = get hyperthermia
posterior - regulates hypothermia
lesion = get hypothermia
paraventricular (magnocellular), supraoptic - ADH, oxytocin - [subforniceal organ senses volume] ->secreted from posterior pituitary/neurohypophysis from neuroectoderm
123
Panayiotopoulos syndrome vs cyclic vomiting syndrome vs SELECTs
occipital epilepsy, see elemental shapes + nausea, vomiting, tachycardia, tachypnea
4-8 years
EEG: high voltage occipital spikes
Don’t need Tx but use carbamazepine
vs Gastaut-type epilepsy - last onset childhood occipital - usual present ~ 8 years, + post ictal migraine
cyclic vomiting syndrome: clusters of vomiting q2-4 weeks, association with migraine HA - Tx sumatriptan abortive; amitriptyline if kid > 5 yrs, cyproheptadine if <5 yrs
-
**SELECTS - facial parethesias, jerking face, hypersalivation**- centrotemporal spikes - age 4-11 yrs
124
Radial neuropathies
Wartenberg - superficial radial nerve-sensory - compression watch/handcuffs - parthesias dorsolateral hand
-motor intact
posterior interosseus neuropathy - **weakness ulnar wrist extension; radial wrist extension spared**, sensory spare
vs. ischemic monomelic neuropathy - AV shunt placement; weakness/sense loss in multiple distributions, radial pulse still palpable
125
median neuropathies
pronator teres syndrome - median nerve - pain in forearm, **sensory loss over thenar eminence** (intact in Carpal tunnel);
[supinator is radial]
anterior interossei - weakness, sensory spared - DIPs 2 and 3, flexor pollicus LONGUS, pronator quadratus -“OK sign”
-neuralgic amyotrophy
CARPAL TUNNEL - flexor retinaculum (NC - **prolonged distal latency** + slow CV b/c focal demyelination( if axonal damage, reduces amplitude)
-tinel/phanel/hand raise test
-spares thenar eminence
-palmar mixed study significant different median + ulnar 0.4 ms
-combined sensory index of 1 or more - maximize sensitivity without reducing specificity -different tests anti-orthodromic
median hand - 1/2 LOAF-LBS (flexor pollicis longus+flexor pollicis brevis superficial head)
- lateral 2 lumbricals
opponens pollicis brevis, ABductor pollicis brevis APB, flexor pollicis longus (OK sign)+flexor policis brevis superficial head
NOT adductor pollicis (ulnar); flexor pollicis brevis deep head- ulnar
abductor pollicis longus - radial
126
Hartnup disease
SCL6A19 - chr 5
diarrhea, red flaky skin, agitation ~Niacin deficiency, sun induced rash
-**AR**, defective amino acid transporter - **increase excretion nonpolar amino acids=tryptophan + phenylalanine in urine**
-no tryptophan absoroption -> niacin precursor
127
femoral nerve
motor: hip flexion, knee extension
anterior femoral - sartorius, pectinus
posterior femoral - rectus femoris, vastus
-above inguinal ligament - ilacus weakness
below inguinal ligament - spared (iliopsoas) - hip flexion
sensory above knee: medial thigh
sensory below knee - saphenous - medial lower leg, foot
128
lateral femoral cutaneous
sensory only - anteriolateral thigh
from L2-L3 directly NOT from femoral
-no w/u if patient presents with this only
129
post stroke fatigue vs depression
fatigue: can use adrenergic/dopaminergic - modafinil
-depression only in late stages
-no associated with sex or age
depression: Beck depression inventory quantitative over time
post-stroke depression + DM2 - pioglitazone effective, unknown MOA
-**SSRIs: risk spontaneous ICH**
-Barthel index - ADLs+mobility, sensitive to psychological factors on recovery
130
OCD Tx kids
SSRI first line; clomipramine (TCA)
Dx - compulsions or obsessions or both
131
motor evoked potential
CONTRALATERAL primary motor cortex
-CST pathway
132
glioblastoma multiforme + anaplastic astrocytoma
Choline peaks > NAA peaks in gliomas (reversal of Hunter peak)
Cho>Cr>NAA
**GBM: choline> lactate > NAA**
-lactate high in areas of necrosis
normal = NAA peak highest
NAA = neuronal integrity
choline - cell turnover
creatine - energy stores (depleted in tumor)
-lipid peak = cell breakdown
anaplastic astrocytoma - nuclear atypia, pleomorphism, increase cellularity
133
Trigeminal nerve anatomy - inferior alveolar
-Ophthalmic-
nasociliary - nose senes
-Mandibular-
1. inferior alveolar - branch CN V3 mandibular
-dental nerve block
2. mental-branch of mandibular - chin, lower lip
3. lingual - anterior 2/3 tongue
4. buccal - (that) inner cheek
134
developmental venous anomalies
caput medusae
135
nucleus ambiguus vs solitarius
ambiguus - CN IX, X - motor only - larynx and pharynx muscles - special visceral efferents pharyngeal arch; and CN VII - stylopharyngeus
solitarius - a Singular Sensation - taste sensation - 7 (chorda tympani anterior 2/3), 9, 10 (epiglottis)
-autonomic nuclei-solitary nucleus
-general visceral afferents: carotid body/sinus - CN 9; SA node/atria CN X
136
brain death examination
no spontaneous breathing/chest wall movement -> apnea test
-if fail apnea test AKA have spontaneous breathing, not consistent with brain death
-need SBP >100, temperature >36
**HIE: 24 hours** or 24 hr after rewarm
-don’t need neurologist
ancillary: transcranial doppler - small systolic peaks, no diastolic flow
-absent BL N19-P22 cortical potentials with median N stim
-brainstem auditory evoked potentials: absence brainstem responses waves III-V with preserved cochlear response wave I
-absent blood flow on MRA
-EEG (old now) - 10 nin, 2 microV
137
autoimmune autonomic impairment
ganglionic nicotinic ACh R
-severe autonomic Sx, dilated pupils
-associated with SCLS paraneoplastic
-subacute <3 months Sx
Tx: plasma exchange, IVIG, but poor prognosis
vs HSAN - sensory deficit
HSAN3- Riley Day- AR
138
Waardenburg syndrome DDx
Waardenburg - AUTOSOMAL DOMINANT - **warlock with a white forelock**, deafness
-defect NCC migration
vs Legius (like NF1 but no Lisch nodules) vs
**Incontinentia pigmenti -, vesicular lesions/pustules**
vs Noonan with multiple lentigines (brown spots)- LEOPARD - electrocardiogram conduction defects, ocular hypotelorism, pulmonic stenosis, abnormal genitalia, growth retardation, deafness
139
axillary vs suprascapular
POSTERIOR cord
-Axillary N - C5-C6 --delt, teres minor (external rotation); triceps long head
-sensation shoulder
-anterior shoulder dislocation
-TERES MAJOR-lower subscapular nerve- C5, C6, and C7 nerve roots- internal rotation
-SUBSCAPULARIS - superior+inferior subscapular N
-thoracodorsal - latissimus dorsi - shoulder adduction
__________________________________________________
UPPER trunk
suprascapular: off upper/superior trunk plexus
-scapular fracture, ganglion cysts, overuse, Parsonage turner/neuralgic amyotrophy/brachial neuritis
-supraspinatus - first 15 degree shoulder abduction with internal rotation (empty can test) + infraspinatus - external rotation
140
Burst suppression DDx
Otohara - tonic seizures - see abnormality on MRI
infantile onset epileptic encephalopathy - STXBP1 - syntaxin binding protein
-infantile spasms
hypasarrhythmia, burst suppression
141
Hyperexplexia
GLRA1 - Chr5
-inhibitory glycine R
hyperkinetic movement disorder - nocturnal myoclonus, exaggerated startle, stiffness , NO seizures
142
AC reversal
warfarin - FFP, vit K (warfarin in rat poison)
andexanet alfa - DOACS
heparin - protamine supfate
**idarucizumab - dabigatran**
143
Medial cord, Ulnar N. vs C8 radic
Ulnar aspect arm: medial
medial cord C8-T1: medial cutaneous nerve of arm + forearm both come off medial cord
C8 radic - (exit C8/T1 marking where nerves exit below vertebrae) normal SNAP
medial forearm sensory loss
lower trunk - abnormal sensory response
144
musculocutaneous N, lateral cord; arm sensation
lateral cord gives off lateral pectoral nerve
-musculocutaneous N -lateral antebrachial nerve branch-lateral forearm ) -
lateral arm sense - inferior lateral cutaneous nerve - radial N branch
medial arm sense is medial antebrachial off medial cord
-median nerve with medial + lateral cord
145
Landau Kleffner
acquired epileptic aphasia
**ESES-electrical status epilepticus sleep** continuous BL centrotemporal spike wave discharges in nonREM sleep
Tx-VPA, diazepam, steroids
(vs. Benign rolandic epilepsy - unilateral centrotemp discharges)
146
vagal nerve stimulation
indication: **drop attacks** (also callosotomy for atonic seizures),
Rasmussen encephalitis, Sturge Weber, lesional epilepsy, MTS, myoclonic seizures
-goal to decrease seizures by 50%
-Treats depression, (migraine, cluster HA)
-takes 1 yrs to work,
-Side effects: hoarseness/voice changes in 2/3 pts (transient); cough
ck battery q6 months
147
Frey Syndrome
auriculotemporal syndrome/gustatory sweating
sweat, flush cheek when eating in auriculotemporal nerve distribution
parotid injury -> no sympathetic cholinergic fxn for sweating -> aberrant regeneration PARAsympathetic fibers
-> paradoxical sweating with parasympathic stimuli - chewing
148
Primary neurulation
Day 24-26
-**closure anterior neuropore -> lamina terminalis** -> anterior wall 3rd ventricle
-anencephaly if fail
149
duchenne, becker muscular dystrophy
XLINKED RECESSIVE
Sx: no reflexes
Tx: steroids first line; deflazacort
etiplersen - ASO antisense - oligonucleotide - restores dystrophin if mutation in exon 51 (~14% pts)
idebenone - co Q10 analong - RESpiratory function (edaravone - ALS)
150
dural AVF vs AVM vs DVA
dAVF - no nidus - direct artery to venous sinus
-acquired
AVM - nidus - flow voids T2 dark
-abnormal connection btw arteries + veins
DVA: irregular arrangement small veins draining into larger vein - “spokes on a wheel” - not danverous
cavernoma - collection of abnormal dilated blood vessels - popcorn - usually asymptomatic
151
carotid artery dissection, Horner's
-resolution in 3-6 months
-usually no repeat recurrence unless risk factors
-HA improves after 1 week
-partial horners, no anhidrosis if ICA dissection b/c superior cervical ganglion at carotid bifurcation
-oculosympathic pathway (pupil constriction)-superior cervical ganglion: ascend via ICA to cav sinus, pupil dilator, Muller muscles
-no response to ciliospinal reflex: painful stimulus to upper cervical/facial area -no expected pupil dilation-
152
Hemiconvulsion-hemiplegia epilepsy syndrome
prolonged focal motor seizure during febrile illness
-acute cytotoxic edema on MRI
vs. Rasmussems - focal epilepsy, prodrome mild hemiparesis, isolated seizures, epilepsia partialis continua
153
Bruxism
-serotonin antidpressants risk of bruxism
-anxiety
-incidence peaks childhood
Tx: can use clonidine, clonazepam but not that effective
154
heritable dystonias
Segawa - Dopa responsive dystonia - GCH1 (GTP cyclohydrolase) deficiency - AD -
("pregnancy HCG movement disorder good prognosis”)
-tyrosin hydroxylase deficiency
-foot dystonia in kid, diurnal fluctuations, parkinsonism later
-focal to generalized: more likely if young kid first in leg than in arm
-geste antagonist in 60%
Generalized dystonia - Torsin gene DYT-TOR1A - AD
-not DA rseponsive
-trihexyphenidyl, tetrabenazine
Paroxysmal kinesigenic dyskinesia - episodic chorea, dystonia from voluntary movement - PRRT2 gene - proline rich transmembrane
rapid onset dystonia-parkinsonism - ATP1A3 gene - segmental dystonia worsening over hrs-days after emotional/physical trigger
(ATP1A2- familial hemiplegic migraine 2 without cerebellar Sx)
Spasmodic dysphonia - "whispering dystonia" - TUBB4 - characteristic facies, body habitus
155
DDx infant hypotonia
Leigh syndrome - subacute necrotizing encephalomyelopathy
-hyperreflexia
-**microcephaly**
-no tracking ->cortical visual processing problem
-still antigravity in limbs
-ophthalmoplegia
-mitochondrial disease can present at birth (vs. Dravet - 6 months)
vs spinal musuclar atrophy - DTRs down, no microcephaly
vs myotonic dystrophy - weaker, DTRs absent
156
myasthenia gravis drugs worsen
CCB
fluroquinolones, Bblockers, aminoglycosides, clindamycin, vancomycin, procainamide, quinidine, anti-PD1 monoclonal Ab (nivolumab), Botox, penicillamine
157
special visceral effect vs general somatic efferent vs general visceral efferent
special visceral efferent - CN V, CN VII, pharyngeal arch derived muscles mastication, facial expression, IV, X palate pharynx, larynx
general somatic efferent - somatic mesoderm - voluntary muscles - intrinsic + extrinsic tongue muscles - hypoglossal N - (from somites not pharyngeal branches)
general visceral efferents -autonomic muscles - orthostatic hypotension response
158
episodic ataxias
Autosomal dominant
type 1 - facial myokymia - KCNA1
-acetazolamide, carbamazepine
“ Eat 1 KFC and wipe grease off my face [facial myokymia]”
EAT2 - CACNA1A - MOST COMMON - brainstem symptoms - triggers stress, EtOH - nystagmus, dysarthria
acetazolamide (eat 2 much calcium and choke)
159
autonomic dysreflexia
bowel/bladder stimulus ->sympathetic response ->vasoconstriction -> HTN -> HR slowed
-if T6 and above
160
delayed sleep wake phase disorder
10% insomnias, FHx
-2 hours before falling asleep - melatonin secreted later
short wavelength blue light bad ?
161
hereditary aneurysms vs AVM vs hemangioblastoma
hemangiomblastoma - VHL - RCC + phenochromocytoma
aneurysms, dissection - Marfans
AVM - Osler Weber Rendu - hereditary hemorrhagic telangiectasia - AD
162
oculopharyngeal muscular dystrophy
GCN/GCG expansion, PABPN1, Chr 14
French Canadian
-ptosis, dysarthria, EOM, facial weakness
163
serotonin syndrome vs NMS vs sympathomimetic vs anticholinergic
**serotonin syndrome: HYPEReflexia, increased bowel signs, mydriasis** low urine OP, coagulopathy
Tx - charcoal if within hrs; cyproheptadine (5-HT antagonist)
**NMS - lead pipe rigidity, normal or decreased reflexes; mydriasis or normal pupils; normal bowel sounds** - antipsychotics
-cause: central, peripheral acute DA block- risk in men who recently increased antipsychotic
-Tx - benzos, bromocriptine, dantrolene (ryanodine receptor antagonist)
sympathomimetic: withdrawal or abuse -reflexes NORMAL, normal bowel sounds; mydriasis
- Tx charcoal, whole bowel irrigation
anticholinergic - dry, mydriasis
malignant hypothermia - normal pupils , normal bowel sounds, hyPOreflexia
164
neurosarcoid
thick patchy leptomeningeal enhancement
-**hypothalamic inflammation ->neuroendocrine dysfxn** -> polyuria, polydipsia, sleep, appetite, temp, libido
-pituitary inflammation
-inflammatory myelopathy
-peripheral neuropathy-small fiber, mononeuritis multiplex, aspetic meningitis
-optic neuropathy
165
XL adrenoleukodystrophy
ABCD1 - LOF ALD protein
-earliest signs behavior changes + MRI involvement -> stem cell transplant (even if older boys)
-no stem cell transplant if no MRI involvement b/c not all have CNS disease
-posterior ventricular white matter disease
Lorenzo oil - lowers very long chain FA concentrations - not effective
166
Reflex epilepsy
reflex = provoked by specific stimulus -
-eye closure sensitivity
1. photosensitive epilepsy - sunflower
-Most common
2. orofacial reflex myoclonic - talking/reading
3. praxis induction - motor tasks - muscle jerks
4. musicogenic epilepsy - music provokes seizures
Mostly GTCs
tx - VPA, LTG, benzo
167
lumbar plexopathy vs femoral neuropathy
lumbar plexopathy: weak thigh adductors, absent saphenous sensory
-retroperitoneal hemorrhage
*normal paraspinals*
Femoral neuropathy: thigh adductors spared
radiculopathy: abnormal paraspinal response;
-neck pain + loss reflexes
-sensory noral
L2-L3 - lateral femoral cutaneous nerve
S1-S2 - piriformis
168
CME vs self-assessment
**Self assessment/self-audit : defined quality metrics** - to stay up to date on standard of care
-compare your practice to standard of care on multiple quality metrics
CME: limited applicability to your practice
board exam - no regular ongoing feedback
169
SSRI side effects
RCVS - SSRI, SNRI adjustments
bruxism
170
Ataxias with vitamin e deficiency
Ataxia with vit E deficiency - low alpha-tocopherol -
-diet or AR mutation TTPA (vit E to liver)
Tx: ORAL vit E (not IV b/c good absorption)
Abetalipoproteinemia - AR - MTTP
-acanthocytosis, multiple DEAK deficiencies
-low total cholesterol, low LDL
Tangier - ABCA1 casette protein -
-low total cholesterol, low HDL
A healthy tangerine with low cholesterol, high TGs; orange tonsils
-can mimic syringomeylia
histo: foamy macrophages
vs, pyruvate dehydrogenase deficiency - episodic ataxia, dystonia, lactic acidosis - infancy to childhood
171
tic paralysis
Dermacentor ticks
can develop over days
-inhibition ACh release after tick feeding for days
171
Midline defects
holoprosencephaly - failure prosencephalon divide
Schizencephaly - migration/organization-1st trimester problem -lined with gray matter
(porencephaly - line with white matter)
hydranencephaly - acquired infectious/vascular insult last 1st/2nd trimester- hemisphere replaced with CSF, only brainstem + cerebellum left
Polymicrogyria - 2nd trimester - problem organization
focal cortical dysplasia - organization problem
172
retinoblastoma, Li Fraumeni chromosome
retinoblastoma - Chr 13 - can have stabismus, glaucoma, nystagmus, proptosis, anisocoria (Not corneal arcus)
Li Fraumeni - Chr 17
172
Kyphoplasty
Tx pathologic vertebral body fractures, compression >50% (mets to spine)
Balloon assisted vertebral body height restoration + cement augmentation
173
EOM palsies anatomy, trochlear nerve vs nucleus
Superior orbital fissure - unilateral CN IV, III, VI
CN IV: **BL trochlear N palsy at superior medullary velum** - decussates in medullary velum as exits from dorsal midbrain
-innervates superior oblique
-alternating hypertropia = R hypertropia with L gaze, etc
-unilateral trocholear nerve palsy (SO-no depression, intorsion, adduction eye): CONTRALATERAL TILT of head to compensate
-worse in contralateral gaze, ipsilateral head tilt
(ipsilateral CN IV nerve or contralateral CN IV nucleus palsy)
Petrous apex: Gradenigo syndrome - Cn VI palsy, facial pain, CN V1 sensory loss
ponto-medullary junction - CN VI palsy
174
nervus intermedius neuralgia
stabbing pain deep in ear CN VII - intermediate nerve of Wrisberg branches off facial nerve at geniculate ganglion (same as Ramsay Hunt)
Tx: carbamazepine
175
Friedrich’s ataxia vs CMT, DDx
**Friedrich’s ataxia - loss sensory potentials, normal motor CV
-DIABETES** - unique to FA
-hunchback, levoscoliosis, pes cavus
CMT 1A - slow CV
EM: no conduction block like CIDP (conduction block=no response on NC stim at proximal site and response at distal site)
CMT1B-myelin protein zero (MPZ) mutation
CMT 2: low CMAP and sensory amplitudes
-reduction myelinated fibers, onion bulbs from Schwann cell proliferation
CMT2-MFN2 mitochondrial fusion protein mitofusin 2 (like MFM=adult=axonal)
X linked CMTX-GJB1
giant axonal neuropathy - curled hair, walk insides feet, childhood distal sensory/motor neuropathy
-mutations in gigaxonin
176
INO - what side is lesion compared to nystagmus?
One and a half vs INO vs PPRF
Eye that can abduct with nystagmus: contralateral to lesion side
Eye that cannot adduct: ipsilateral to lesion side
One and a half syndrome: pons tegmentum - PPRF (no eye movement when looking to lesion side), MLF
just PPRF lesion: can’t look ipsilateral to lesion with either eye + coarse end gaze nystagmus looking contralateral
177
Coccidioidomycoses
-subacute meningitis, southwest US
Tx - fluconazole even for meningitis
Dx: CSF coccidioides antigen + PCR testing
178
Alcoholic hallucinosis vs delirium tremens
**Seizures: 6-48 hours**
[Hallucinosis: 12-48 hours - visual+tactile hallucinations, preserved orientation before DTs]
DT: 48-72 hours - confusion + hallucinations
179
vertebrobasilar ischemia
orthostatic hypotension with new BP meds
-vertigo, drop attacks, perioral numbness
->compression vertebral artery - bone spurs
-comparative BP measures standing/sitting and auscultation neck-> to check for subclavian steal
180
percentage sleep stage by age
**N2>REM>N3 slow wave**
N2 is 50% of sleep for all ages
-REM suppressed by meds
younger: more N3, more REM, less N2
older: more N2, less N3, less REM
narcolepsy - REM early in sleep
181
prosopagnosia, Balint
bilateral fusiform gyri (vs temporal-occipital - difficulty with object recognition)
Balint - bilateral parieto-occipital lobes
medial occipital - Anton syndrome - cortical blindness - NO optokinetic response
182
atonic seizures exacerbate, Tx
exacerbate: carbamazepine, phenytoin
rufinamide just for LGS- risk short QT syndrome
183
Takotsubo
ICH, stroke, SAH
-can have ballooning outside the apex
-normal cardiac cath
184
Kennedy disease X linked spinobulbar muscular atrophy weakness pattern vs ALS
proximal > distal weakness, LMN signs only
-Diabetes
X linked CAG repeat in androgen R gene
-perioral/**facial twitching** (may not give gynecomastia)
EMG: diffusely large motor units (reinnervation)
**absent sensory responses**
vs MMN-GM1 Ab-pure motor problems
185
ASMs avoid in mitochondrial disease
carbamazepine, phenobarbital, VPA, phenytoin
186
Diphtheria mechanism arrhythmia
cardiac vagal denervation -> parasympathetic dysfxn
abnormal bradycardia/valsalva response
187
absence epilepsy
20 sec or less, no postictal confusion
188
SAH management
Noncommunicating hydrocephalus -> EVD (lumbar drain for communicating hydrocephalus)
no hyperventilation b/c vasoconstriction -> vasospasm
vasospasm peak 5-7 days
189
Coital HA Tx
indomethacin
190
medial, lateral pectoral nerve
medial pectoral - off medial cord C7,C8 / C8/T1
-medial brachial, medial antebrachial
pectoralis minor and the sternocostal head of pectoralis major
lateral -off lateral cord
191
oculocerebrorenal syndrome / Lowe
OCRL1 gene
X linked
-cataracts, glaucoma
-renal failure
-areflexia, hypotonia
192
kluver-bucy vs witzelsucht
Witzelsucht: inappropriate jocularity - OFC also
(OFC also -antisocial)
amgydala - Kluver Bucy
gegenhalten - paratonia
ganser syndrome - dissociative disorder
193
cervical myelopathy Sx + indicators poor prognosis
**Symptoms > 18 months**
Age, female
*urinary symptoms*
Asian -post. long ligament ossification
fasciculations only in arms (C5-C7) vs ALS
-EMG with changes only in UE
[primary lateral sclerosis - only UMN]
flail arm ALS - brachial amyotrophic diplegia - proximal arm, asymmetric, spreads distally
cervical roots exit above, thoracic roots exit below
194
spinocerebellar ataxia
SCA1 - CAG repeat ATXN1 gene chromosome 6
-SCA3: Machado joseph- most common - CAG ATXN1 gene
SCA 7 - teenage, early adult - retinopathy
autosomal dominant
195
ulnar N, Froment sign
Proximal to Guyon Canal:
forearm: flexor carpi ulnaris, flexor digitorum profundus 4, 5 (flexes DIP);
-flexor carpi ulnaris
-**palmar cutaneous nerve - sensation over hypothenar eminence**
Distal Guyon Canal:
-4th + 5th lumbricals - flex at MCP
-adductor pollicis - thumb ADDuction weakness
Isolated loss of the 5th digit ulnar SNAPs could be seen with an injury distal to Guyon's canal and proximal to the 5th digit. A
at guyon canal - **Froment sign - flex thumb to compensate for adductor pollicis weakness**
-dorsal, palmar interossei- finger adduction/abduction at MCP
- flexor pollicis brevis deep head - thumb flexion at MCP
mid-palm injury: isolated involvement FDI CMAP as this would spare the branches to the hypothenar eminence and the digits.
hypothenar muscles (abductor digiti minimi)
196
Tx HTN acute stroke
Risk with home med and TPA?
NiCARDipine CCB, clevidipine, labetalol
**ACEi increase bradykinin -> increases risk orolingual angioedema** bc TPA increases bradykinin via thrombolysis
197
polymicrogyria, lissencephaly genes
LIS1 - miller dieker - chr 17 - 4 layer classic lissencephaly -
-usually sporadic LIS1 deletion; if familial is AD b/c lose one copy of LIS1 on Chr 17 and get disease
Type 1 - facial dysmorphism, 17p deletion
type 2 - hydrocephalus, dysgenesis cerebellum - Walker Warburg
DCX - x linked lissencephaly - doublecortin
female: subcortical band heterotopia (have all 6 layers)
Periventricular nodular heterotopia- X linked FLNA gene - cytoskeleton stabilization - females X linked dominant
Polymicrogyria: CMV
-GPR56 gene
Zellweger’s
198
grading germinal matrix hemorrhage
Premies <32 weeks or low birth weight, germinal matrix bleeding btw caudate + thalamus
I: blood at germinal matrix - good prognosis
II: blood fills ventricles but normal size - good prognosis
III: blood dilates ventricles - poor prognosis
IV: parenchymal hemorrhage from venous infarction - poorest prognosis (dilated ventricles compress veins)
199
Embryonal tumors
aggressive, grade IV
1. Medulloblastoma
2. Embryonal tumor with multilayered rosettes - ETMR + C19MC
-pseudostratified epithelium with central clear lumen - heterogenous contrast enhancement, **diffusion restriction**
-frontal, parietotemporal
-ages <5
-C19MC amplification (non-medulloblastoma)
3. if no C19M classification but has pseudostratefied neuroepithelium -> medulloepithelioma
4. atypical teratoid/rhabdoid - INI1 loss - inactivation sMARCB1 on Chr 22 or BRG1 loss
Tx: resection, chemo +/- radiation
200
visual evoked potentials MS - what latency is abnormal? where generated? Optic neuritis on OCT
p100 prolonged 120 msec in bad eye
-VEPs sensitive not specific for optic neuritis
from occipital cortex- striate + prestriate
OCT: retinal nerve fiber atrophy associated with cerebral atrophy, disability, decrease visual function (increase fiber layer = edema)
201
wernicke korsakoff
Sx: can be VERTIGO or HEARING loss, not always EOM abnormality and gait ataxia
-peripheral nerupathy - LE
-pts with cancer
Damages anterior nuc thalamus in Papez circuit
Hippo ForM ANT CEH
hippocampus to fomix to mamillary body -> mamillilothalamic fibers - > ANT -> cingulate -> entorhinal -> hippocampal formation
Korsokoff - antero+retrograde amnesia - MEdial temp lobes
202
cerebellum structure
3 layers: molecular stars in a basket, Purkinje, granular ground of sparks
Superficial layer - Molecular - stellate, basket - inhibit purkinje cells
Middle - Purkinje - inhibitory cells
Deep layer - Granular cell - excitatory
all neurons INHIBITORY except granular cells
203
polymyalgia rheumatica, giant cell / temporal arteritis
5-30% get temporal arteritis
-swollen chalky white optic disc
-**Most common Sx: HA, shoulder/pelvic girdle pain, fatigue/malaise**; jaw claudication in 40%, less likely fever
biopsy: mononuclear infiltrate, intimal thickening + luminal stenosis
doppler ultrasound: halo sign (hypoechoic around artery 2/2 edema)
Tx: steroids; long term Tx Tocilizumab - anti-IL6
-give calcium + vit D b/c chronic steroids
204
wilson
ATP7B
Kayser fleisher - limbus of cornea
-urinary copper high
205
familial transthyretin amyloidosis
AD, TTR-mediated amyloidosis - pattiesserie -
patisiran, inotersen, vutrisiran - inhibit hepatic synthesis TTR
-Tx peripheral neuropathy
tafamidis - cardiomyopathy
206
Bickerstaff vs autoimmune encephalitis vs Susac
bickerstaff - bilateral ophtahmoparesis
susac - autoimmune vasculopathy - thromboses brain, retina, inner ear
-branched retinal A occlusions
-MRI: holes in corpus callosum
207
C6 vs C7 radic
C6 - thumb and 1st digit sensory loss, lose biceps and brachioradialis reflex
C7 - middle finger sense loss, triceps weak
208
brain iron accumulation
dytonia , movment disorders
vs. hepatolenticular degeneration - Wilson’s
209
personality disorders histrionic vs borderline ; OCPD vs OCD
cluster b - dramatic/emotional/erratic- antisocial, borderline, histrionic, narcissistic
histrionic - hypersexual, perceives relationships as more intimate than they are - tearful when denied something
borderline - violent - splitting when denied something
narcissistic - when denied, anger, aggression
OCPD.- miserly, rigid, control issues,
210
cerebral amyloid angiopathy
APO E gene mutations
211
KRIT1
hereditary cavernomatosis
-recurrent hemorrhages + headaches
(aunt chris)
212
critical illness myopathy histo
myosin loss
213
donepezil
prolonged QT syndrome
214
toluene vs heroine vs ketamine vs NO, vs Curare
toluene: euphoria, hallucinate, depression, nystagmus, ataxia, turn skin grey
inhaled heroine - chasing the dragon - ideomotor apraxia, depression abulia, leukoencephalopathy
-Tx opioid withdrawal-mu opioid agonist - methadone, buprenorphine.- partial mut R agonist-agonist
-precedex - clonidine - alpha 2 agonist (blocks release NE, suppresses autonomic Sx of withdrawal)
(midodrine alpha1 agonist)
NO - laughing gas - NMDA antagonist
-precipitate b12 deficiency
Curare poisoning- mimic locked in - paralysis skeletal muscles
215
brown sequard
ipsilateral Horner’s
-ipsilateral vib, proprio loss, strength loss
-contralateral pain temp loss
216
REM parasomnias
REm sleep behavior
nightmares
sleep related graoning
217
grading concusion, diffuse axonal injury
concussion - no LOC
cerebral contusion - <6 hr LOC
grade 1 diffuse axonal injury - mild - microscopic
Mild DAI: LOC <24 hr, chance good recovery 60%
grade 2 - focal lesions **corpus callosum**
moderate DAI: LOC >24 hrs, good recovery at 3 months 40%
grade 3 - **brainstem** microhemorrhages
severe DAI - LOC for days chance good recovery 15% at 3 month
-decorticate posturing
218
paraneoplastic ab
parkinsonism-BG-
anti-CRMP5, Ma2, NMDAR,GAD-65
anti-Ta (Ma2) - limbic encephalitis -testicular cancer
-medial temporal lobe hyperintensity
MA will point out the 5th limb (LIMBIC)(elephant penis) next to testicles
SCLC:
- CRMP5 - limbic encephalitis ; cerebellar degen.
-Anti Hu - peripheral neuropathy ; encephalitis
-ACHR-R nicotinic Ab - autonomic Sx, neuropathy- dilated pupils
Isaac syndrome - voltage gated potassium channel - neuromyotonia
-carpopedal spasm, sweating
opsoclonus: anti-Ri - neuroblastoma
You’re anti Rihanna- dancing eyed Jerk!! Blast your neurons!
anti-synthetase syndrome - anti-Jo-1-mechanic’s hands, Raynaud’s
anti-synthetase-anti-PL7/PL12, OJ, EJ
dermatomyositis - Mi2,
TIFI-y, NXP-2-cancer
MDA-5-ILD
-screen CT abdomen/pelvis
“polymyositis"-ILD-CT chest, elevated aldolase
immune mediated necrotizing- super high CK
anti-SRP (myocarditis), anti-HMG-CoA reductase, antimitochondrial
219
aseptic meningitis infectious cause Mollaret VS VZV
Mollaret - HSV 2 - 3 episodes of fever + meningismus, lymphocyte predominant
- don’t need to have genital lesions
Dx: HSV 2 PCR in CSF; high lymphocyte WBC, ~normal protein, glucose, no RBCs
vs HSV1-MCC fatal sporadic viral ENCEPHALITIS (hemorrhagic temporal lobe)
-consider NMDA R encephalitis if worsen after HSV-1 encephalitis
vs VZV - vasculitis, SAH, aneurysm
220
glossopharyngeal neuralgia
**Bradycardia**; rare asystole, syncope
-pain at angle of jaw, throat, ear, base of tongue
-triggered by swallowing/yawning
- CN IX into tractus solitarius effect on CN X?
221
alexia without agraphia vs alexia + agraphia
alexia without agraphia: corpus callosum lesion, spare angular gyrus
alexia + agraphia: angular gyrus lesion
222
VZV vasculopathy
cause: vasculitis large and small arteries, SAH, aneurysm
Dx: VZV IgG Ab in CSF
223
Aromatic acid decarboxylase deficiency
AR
-makes DA, epinephrine, NE, 5HT
-Sx: 1st yr life, axial hypotonia, appendicular hypertonia, delay, **oculogyric crisis, autonomic crisis***
-Sx worse later in day
-build up Dopa and 3-OmethylDA in blood
-Tx: Da agonist
-usually misdiagnosed
224
dialectal behavioral therapy vs metacognitive therapy
metacognitive - look at thought patterns leading to actions
beliefs about your thought content rather than content itself
vs dialectal - treat emotional reactions, relationship difficulty
225
neurohypophysis vs adenohypophysis
neurohypophysis: posterior pituitary - neuroectoterm - secretes ADH, oxytocin make from paraventricular/suprachiastmatic hypothalamic neurons
adenohypophysis - anterior pituitary - Rathke pouch
226
most common to least common hypertensive hemorrhage pons, cerebellum
putamen (50%), thalamus (15%), pons then cerebellum
227
cerebral palsy hemiplegic vs spastic quadriparesis
hemiplegia - perinatal stroke
spastic quadriparesis, dyskinetic CP - perinatal hypoxia
-static disorder - unlikely to progress
228
Dravet treatment stiripentol
stiripentol + clobazam - if 6 months
-SE: somnolence, anorexia
stiripentol - GABA R direct allosteric modulator - decrease cell injury after status? neuroprotective?
-inhibits metabolism of clobazam, valproate
229
CMV path vs Rabies path
Owl eye - basophilic inclusion body
-cause: retinitis
Tx: ganciclovir
Rabies: negri body (dark spot in periphery of cytoplasm - lighter than inflammatory cells)
230
ADEM
monophasic
-young adult after infection
-CSF normal or WBC/protein elevated
231
decort vs decerebrate
decorticate: at red nucleus - : disinhibit red nuc, activate rubrospinal tract -> flexor in uppers
decerebrate: between lateral vestibular nucleus (Deiter-caudal pons-maintains upright posture) and red nucleus b/c no inhibition of extension caused by vestibular nucleus
vs injury upper cervical spine/cortex = no posturing
232
SUDEP risk factors
highest risk 18-40
kids: if frequent GTCs at night can use nocturnal supervision Or remote listening device
232
more common - hyper or hypokalemic PP?
HYPOkalemic PP - most common -CACNA1S usually or SCN4A
-trigger: carbs, exercise
hyperkalemic PP - SCN4 (can get paramyotonia congenital)
-trigger: fasting, exercise
233
herniation syndromes
uncal - ipsilateral blown pupil, contralateral hemiparesis OR ipsilateral weakness (Kernohan notch- contralateral peduncle compressed)
subfalcine - leg weakness 2/2 ACA trapped under falx + hydrocephalus
ascending transalar - temporal lobe herniates up across sphenoid ridge; can get MCA / ACA infarct - middle cranial fossa lesion
tonsillar - cushing triad
234
junctional scotoma localization
optic N + willebrand knee - contralateral nasal retina fibers -> monocular scotoma + contralateral superior temporal field loss
scotoma of Traquair - monocular temporal hemifield cut from ipsilateral nasal retinal fibers anterior to optic chiasm
235
jugular foramen nerves compression syndromes
Vernet: CN 9-11 - intracranial lesion compressing (Vascularis 10, 11 and Nervosa 9)
Collet-Sicard: CN 9-12 - tongue deviate, atrophy - extracranial lesion invading
Tapai: CN 10, 12, 11, Horners - oral intubation, mets, carotid dissection
pars nervosa-9, inferior petrosal sinus
pars vascularis - jugular bulb - 10, 11
pars vascularis - **jugular bulb, internal jugular vein, CN X, IX, ** auricular from X, post. meningeal branch ascending pharyngeal A
236
cortical basal degeneration (astrocytic plaques) vs PSP (tufted astrocytes) vs MSA (glial inclusions)
CBD: neuroimaging MOST helpful to Dx - asymmetric atrophy frontoparietal
-4R hyperphosphorylated tau in neurons + glia, **astrocytic plaques**
-widespread neurodegernation, hyperphosphylated tau
MRI: midcalosal atrophy, asymmetric atrophy parieto-frontal cortex
PSP: tufted astrocytes (astrocytes with tau)
MSA: α-syn within oligodendrocytes- glial cytoplasmic inclusions
237
myotonic dystrophy inheritance 1 vs 2
Autosomal dominant
Type 1 - anticipation - CTG repeats in DMPK dystrophia myotonica protein kinase on Chr 19
-posterior subcapsular cataracts
-hypersomnolence, central sleep apnea, baldness, cardiac conduction defect
Type 2 - CCTG repeat on zinc finger 9 on chr 3-autosomal dominant
-no systemic involvement
-no anticipation
238
AIDP with HIV
lymphocytic pleocytosis with AIDP
-AIDP not associated with HIV -> albuminologic dissociation
still get absent F waves
-don’t see viral particles on biopsy - see demyelination
239
progressive encephalomyelitis with ridigity and myoclonus
PERM - encephalopathy
-autonomic instability
usually autoimmune
-West nile/brucellosis post infectious
-thymoma association
multiple Ab pargets-gad 65, glycine, DPPX, amphiphysin
(stiff person syndrome has no encephalopathy
240
cluster HA duration and treatment
15 minutes to 3 hours
-parasympathetic activation causes Sx
Tx: verapamil
add on topiramate
241
congenital myopathies - Nemaline
nemaline - early respiratory failure, face neck flexors, proximal muscle weakness early in disease
242
epilepsy genes
1. Benign idiopathic neonatal convulsion
fifth day fits, seizure 4-6 days
-variant theta rhythm on EEG
-clonic seizures
-KCNQ2
2. Benign familial neonatal seizures
KCNQ2, KCNQ3 gene, AUTOSOMAL DOMINANT
-EEG NORMAL, MRI normal, normal growth/dvelopment
Tx: no tx or oxcarb, carbamazepine
3. benign familial infantile seizures; SCAN2A, SCAN8A - Autosomal dominant
-start at 6 months and seizures end at 2 years
-association with adult paroxysmal kinesigenic dyskinesia in adolescence-PRRT2 gene - proline rich transmembrane protein 2 gene - Chr 16 p
febrile seizures-GEFS+. SCN1A
**autosomal dominant nocturnal frontal lobe epilepsy - CHRNA4 gene** - alpha 4 subunit **nicotinic acetylcholine receptor** - seizures out of SLOW WAVE SLEEP - (CHERNING nicotine) hypermotor seizures
-Tx: carbamazepine
JME - GABRA1 - Gaba A receptor
acquired epileptic aphasia Landau Kleffner - NMDA R
Glucose transport type 1 deficiency - SLC2A1 gene - infancy, seizures, cognitive impairment, motor incoordination
SCN1A-Dravet
pyroxine dependent neonates - alpha aminoadipic semialdehyde dehydrogenase - antiquin
243
tx post herpetic neuralgia
gabapentin, pregabalin
NOT carbamazepine/LTG
244
MS supportive drugs
Dextromethorphan-quinidine - pseudobulbar affect (lack of facial expression)-NOT pseudobulbar palsy
-dextromethorphan = NMDA glutamate R antagonist, 5-HT, NE reuptake inhibitor
-quinidine given to block hepatic metabolism of dextromethorphan
dalfampridine - interacts voltage gated potassium channels
walking in MS
245
reflex bradycardia response sensory
general visceral afferents
246
sleep eeg by age
Spindle-K-Post
2 months - sleep spindles first appear, asymmetric, asynchronous
2 yr - sleep spindles synchronous
3-6 - K complexes, clear sleep spindles
6 yrs - POSTS, 14+6 positive spikes
247
facioscapulohumeral muscular dystrophy
deletion D4Z4 region
-AUTOSOMAL dominant chr 4
-can’t bury eyelashes, scapular winging
-RETINAL Vasculopathy - 50% -
-RESPIRATORY involvement -BL PFTs
-screen hearing loss (common esp if large deletion)
-MSK pain
248
artery adamkiewicz origin
CST lateral vs anterior
T8-L2
anterior CST: axial muscles ipsilateral, MOST ventral, more than anterior spinocerebellar tract
vs lateral CST
249
primary enuresis vs secondary
for kids 5 yrs and up
primary monosynaptic - never achieved dryness and older than 5 yrs -
-2x more common in boys
Tx: motivational therapy, beahvioral interventions, enuresis alarms first line
**high rate spontaneous resolution**
nonmonosynaptic - urinary urgency, daytime incontinence, pain, frequency
250
HIV- HAND
confluent subcortical T2 hyperintensity - no enhancement
HIV associated neurocognitive disorder
-mild subcortical dementia
-psychomotor slowing
251
dengue fever neuro manifestations
rare, 1% of cases
1. transverse myelitis
2. ADEM
3. GBS
mononeuropathies, polyneuropathies
DEN1 Ab
thrombocytopenia
PCR in CSF
252
her
in pts with DM, still exclude other causes, can have additional cause in up to half of pts
most common EMG - distal axonopathy
253
thyroid eye disease
thyroid ophthalmopathy/orbitopathy
TSH-Ab - Graves
proptosis, chemosis, injection to eyes, tearing, conjection, lid retraction, diplopia b/c big extraocular muscles, -> compressive optic neuropathy
less common - TPO Ab - Hashimoto’s
254
monomelic neuropathy vs monomelic amyotrophy vs neuralgic amyotrophy
ischemic monomelic neuropathy - AV shunt placement; weakness not in nerve distribution
monomelic amyotrophy - Hirayama disease - LMN - distal UE weakness - Asian, no sensory Sx
255
pseudodementia memory
spared: implicit memory - Ex riding a bike, making a favorite meal
affects semantic memory, episodic memory, remote + working memory
256
stroke + triptans + MOA
contraindicated
triptan: 5HT1b 5HT1d agonist
257
ventromedial PFC vs ACC
VMPFC: confabulation
disinhibition
emotional lability
ACC - abulia
supplementary motor cortex - mutism, akinesia
amygdala hyperactivity- anxiety - hy
258
IBM Ab
NT5C1A protein autoantibody
259
conductive aphasia
no repetitive
arcuate fasciculus
260
Waldenstrom macroglobulin vs MM vs MGUS vs IgM paraproteins anti-MAG antibodies
waldenstrom - IgM
M spike Multiple Myeloma - IgG
MGUS - IgG or IgA
IgM paraproteins - anti-MAG-distal acquired demyelianting sensory polyneuropathy -acquired stocking gloves and feet neuropathy
261
KCNA1
Mutations in KCNA1 have been associated with a variety of human diseases, including:
Myokymia with periodic ataxia (AEMK)
Episodic ataxia type 1 (EA1)
Neuronal developmental disorders
Cardiac dysfunction
Familial paroxysmal kinesigenic dyskinesia
Malignant hyperthermia (MH)
Epilepsy
Hypomagnesemia
262
unresponsive wakefulness vs minimally conscious state vs coma
minimally conscious: awareness of self or environment (even if inconsistent)
unresponsive wakefulness (vegetative) - spontaneous eye opening, no purposeful behavior
coma - no eye opening no wakefulness
263
primary, secondary vesicles
mesencephalon- midbrain, cerebral peduncle
rhombencephalon = metencephalon (pons, cerebellum, upper 4th ventricle)+ myelencephalon (medulla, lower 4th ventricle)
mesencephalon - midbrain + cerebral peduncle
prosecephalon - telecephalon (hemispheres, BG, lateral ventricles), diencephalon
264
epilepsy with myoclonic-atonic seizures
Doose syndrome - Doose is loose
- age 1-5 yrs
-2-5 Hz spike wave
265
CJD
total tau elevated in CSF (in AD - low amyloid beta, high phosphorylated tau)
-MRI has DWI cortical ribboning - most patients have DWI abnormality
-DWI abnormality - basal ganglia
typical EEG: 1 Hz periodic BL **sharp wave complexes **discharges/sharp wave complexes
-NOT monophasic
266
Thalamic nuclei + hearing
organ of Corti to ipsilateral cochlear nuclei to contralateral inferior colliculus via lateral lemniscus -> BL projections to
medial geniculate nucleus -> superior temporal gyrus aka primary auditory cortex
inferior temporal gyrus - visual processing
superior colliculus - eye movements
267
progressive myoclonic epilepsies
1. Unverricht-Lundborg - EPM1 - (Baltic sea)- cystatin B - chr 21
-generalized discharges
-6-15 yrs, stimulus sensitive myoclonus
2. Lafora -EPM2A deletion - AR - Chr 6
SKIN biopsy -PAS positive intracellular dots
-present 12-17 yrs
-**occipital** spike wave discharges
3. MERRF - mitochondrial
4. neuronal ceroid lipofusinosis - blind, dementia, facial dyskinesias as adult
CLN1-3, PPT1, TPP1 genes
-present any age
5. sialidosis - NEU1 - NEW FARM SILO - cherry red spot - alpha-N acetyl neuraminidase deficiency >mucopolysaccharidosis
- present 1st yrs of life
-Dx: urinary sialyl olicosaccaride, lymphocytic vacuolation
268
inhibitory neurotransmitters of CNS, spinal cord
glycine - principal inhibitory neurotransmitter of spinal cord
-chloride enters, causes inhibitory postsynaptic potential
GABA - principal inhibitory neurotransmitter everywhere in CNS but spinal cord
269
facial dermatomes
v1 - middle of nose
v2 - top lip, lateral nose, - infraorbital, zygomaticofacial, mygomaticotemporal (squidward nose)
v3 - bottom lip -mentalis,
-buccal, auriculotemporal, mental branches
c2-mandible angle, superior occiput like a wrap around the ear - greater auricular nerve
270
leg dermatomes
heel - L5 (sliver of medial heel s2)- 1st, 2nd third toes - L5
big toes, medial leg below knee - L4
pinkie toe , lateral foot- S1
buttocks, post, leg - posterior popliteal fossa - S2
271
antiemetics for migraine
Tx: metoclopramide (Reglan), chlorpromazine (thorazine/1st gen low potency antipsychotic like thioridazine); prochlorperazine (Compazine for nausea)
No evidence for migraine: ondansetron (Zofran)
272
Midbrain syndromes
Weber syndrome- Weak- ventromedial midbrain lesion, ipsilateral cranial nerve (CN) III palsy, contralateral hemiparesis
-motor=medial
Claude syndrome - Clusmy - dorsal midbrain tegmentum lesion, ipsilateral CN III palsy, contralateral cerebellar ataxia, red nucleus
Benedikt syndrome- Both-WEAK + ataxia/tremor; midbrain tegmentum lesion, ipsilateral CN Ill palsy, contralateral hemiparesis, contralateral cerebellar ataxia/ tremor/ choreoathetosis
Dorsal midbrain syndrome, aka Parinaud syndrome: midbrain pretectum lesion, vertical gaze paresis, light-near dissociation, convergence-retraction nystagmus,
Collier lid retraction
273
spinal cord infarct
can start with back pain but is abrupt in onset
T6 above - bradycardia, hypotension, autonomic dysreflexia
sulcocommissural artery-branch of ASA at pons level - anterior CST, reticulospinal
arterial vasocorona - connects ASA/PSA
-Artery Adamkiewicz-C6 and below-bladder incontinence + loss reflexes; dorsal columns spared.
274
toxoplasmosis vs cns lymphoma vs abscess vs tumefactive demyelination
toxo: hemorrhagic, multiple components
**PCNSL: Notch sign **- deep depression at tumor margin when contrast enhanced
-no DWI signal, no hemorrhage, lots of edema
-specific to PCNSL, not in GMS
GBM-DWI signal
abscess: homogenous DWI signal
tumefactive demyelination - little edema/mass effect
275
muscle fiber types + sensory
1a-muscle spindles-**myotatic stretch reflex**-input to alpha motor neurons-reflex
1b-golgi
II-pacisian, meissier -pressure
III—pain+temp STT three letters
C fibers-small unmyelinated - c -pain
gamma motor neurons-innervate **intrafusal** fibers-proprioceptive-muscle stretch
276
Tx for LEMS and MOA
3,4 diaminopyridine-inhibits presynaptic potassium channels
277
botox type and MOA
A, C, E - cleave SNAP 25 (every other)
all the rests - synaptobrevin - B, D, E, F and tetanus toxin
278
organophosphate poisoning
impaired ACHestase functioning
-Tx-atropine - binds competitively at muscarinic R (not nicotinic-skeletal muscle)
279
epimysium vs perimysium vs endomysium
epimysium - around collection of muscle fascicles (epineurium - multiple fascicles)
perimysium - individual muscle fascicles (perineurium - individual nerve fascicles)
endomysium - muscle fibers
280
neurogenic thoracic outlet
medial cord injury
-weaknes to ABP and 1st doral interossei (medial and ulnar muscles mixed) + sensory loss to MEDIAL forearm and hand
vs C6 radic-weakness in the elbow flexors (musculocutaneous biceps)- numbness - lateral hand.
upper trunk lesion would cause weakness in the muscles supplied by the C5/6 roots, including the shoulder and elbow flexors along with numbness in the lateral portion of the arm and hand.
281
L5-S1 paracentral disk hernation
L5/S1 level are likely to compress the S1 nerve root, which will exit at the next level below (S1/2 neural foramina). The S1 root innervates the gastrocnemius (elevation onto the toes) and its fibers are responsible for the ankle reflex (hence, ankle reflex would be absent and not preserved). An L5 radiculopathy would cause weakness of great toe extension (EHL), but the L5 nerve root runs laterally to a paracental disc herniation as it exits the L5/S1 neural foramen and thus would be spared.
The L3/4 roots innervate the knee reflex.
The medial calf is saphenous/L4 territory-femoral
282
suprascapular nerve entrapments spinoglenoid
suprascapular N at spinoglenoid notch - infraspinatus palsy- just external rotation weakness, spared abduction
suprascapular N at suprascapular notch - supraspinatus - extenal sutation + abduction to 15 weakness
283
conus medullaris vs cauda equina
conus - unable to achieve an erection, can’t urinating, decreased sensation around anus but not saddle anesthesia; symmetric heaviness in his legs
cauda equina - saddle anesthesia, PAIN, asymmetric weakness
284
weakness L index finger abduction
-distal wound to mid palm
vs:
At Guyon’s canal- ulnar hand muscles + 4/5 digits sensory loss - hypothenar eminence sense spared
-dorsal ulnar cutaneous spared
ulnar neuropathy at elbow: +dorsal ulnar cutaneous + hand muscle weakness
-lower thoracic outlet-C8/T1 medial cord- ulnar and median innervated hand muscles along with sensory loss in the medial hand and forearm.
-scalene triangle fibrous band
-C8 nerve root- weakness of the radial, ulnar and median innervated hand muscles along with sensory loss in the medial hand and forearm.
285
MCC familial ALS + inheritance
AD AUTOSOMAL DOMINANT
-C9ORF72-20-40% expansion hexanucleotide
-SOD1 point mutations-10-20% of cases
TDP-43 -familial and sporadic ALS -<5% cases
286
foot drop L5 radiculopathy vs peroneal neuropathy
To distinguish:
L5: -hip abduction - L5 nerve root- gluteus medius muscle -superior gluteal nerve
-ankle inversion-posterior tibialis muscle- tibial nerve and supplied by the L5 nerve root (tibial N larger terminal branch sciatic N L4-S3 vs peroneal L4-S2)
[hip adduction obturator L2-L3 adductor magnus]
[peroneal+L5-1st toe extension-extensor hallicis deep fibular nerve, weak in L5 radiculopathy]
L4-tibialis anterior - deep peroneal
only motor branch of common peroneal: short head biceps femoris
L5 nerve root, - gluteus medius; tensor fascia lata (superior gluteal nerve)
To distinguish peroneal vs S1: ankle plantar flexion - S1 tibial
S1 nerve root- gluteus maximus
287
congenital myasthenic syndromes -
AR except slow channel - AD
COLQ- slow/absent pupillary response; EOM abnormalities
288
DADS polyneuropathy
glove and stocking distribution numbness and unsteady, prolonged distal latencies on NC
+anti-myelin associated glycoprotein-
**anti-MAG Ab + IgM paraproteins**
289
L3/4 radiculopathy vs femoral N injury
Hip adduction - obturator - L3/4 action
extension at knee weak
(hip abduction L5)
290
ganglioglioma vs gangliocytoma staining
ganglioglioma: ganglion + glial cells
+GFAP (atrocytes - also GBM) , synaptophysin, CD34 (neuronal)
-temporal lobe
gangliocyoma: no +GFAP staining, only ganglion cells no glial cells
291
motor evoked potentials
stimuli-transcranial magnetic stimulation to primary motor cortex
voluntary contraction-facilitation: increase MEP amplitude, shorter onset latency, prolonged potentials
292
pseudobulbar palsy vs progressive bulbar palsy vs pseudobulbar affec
psuedobulbar palsy: CN dysfunction outside brainstem, UMN pattern signs
progressive bulbar palsy: degeneration neurons in brainstem motor nuclei
pseudobulbar affect - supratentorial difficulty emotional control
293
MRIs and implantable pacemakers
increase serum trop if pacemakesr b/c heating effects-electric currents
-decreases battery life when exposed to magnetic field
-can’t get MRI if DBS, cochlear implant unless MR safe/conditional
294
Perinatal stroke
arterial stroke-most common 1st few days of life - focal seizures
venous infarct - periventricular venous infarct - preterm, in utero
295
aica
middle cerebellar peduncle, CN VII fascicles, hearing loss
296
receptive aprosody localize
difficulty understanding prosody/intonations of speech - right temporoparietal
297
predictive factors of malignant post stroke edema
**DWI with infarct 82 ml** or larger = clinically significant swelling
or NIHSS>15 nondominant and >20 in dominant
298
posterior cortical atrophy
**Tauopathy** with Gerstman syndrome or Balint syndrome, alexia, apraxia, **VF defects**
-episodic memory spared vs Alzheimer’s
MRI - BL but can be asymmetic - parietal/occipital; temporo-occipital atrophy
299
Intraventricular mass DDx
-colloid cyst -MONRO- single layer columnar epithelium, makes mucin, homogeneously enhancing on FLAIR
-Sx: obstructive hydrocephalus
[Monro: btw lateral + 3rd ventricle at level of thalamus]
central neurocytoma - round nuclei uniform cells, small cytoplasm
-lateral ventricles or foramen on Monro
-heterogeneous on imaging vs colloid cyst
choroid plexus papilloma - papillary structure with fibrovascular core lined by cuboidal epithelium
-adults - 4th ventricle
-kids - lateral ventricle
subependymoma - clusters of small nuclei in fibrillary background -scattered microcysts
-4th ventricle, lateral ventricles
subependymal giant cell astrocytoma - TSC - large polygonal eosinophilic cells
(luschka, magendie off 4th ventricle)
300
salivatory nuclei
Pons parasympathetic nuclei
-superior salivatory nucleus (submandibular=submaxillary, sublingual - facial-chora typani carries preganglionic parasympathic fibers) + inferior nucleus (Parotid-otic ganglion-glossopharyngeal)
-stimulated by ACh to produce more saliva
posterior salivatiry nuclesu -
301
nucleus ambiguus - motor nucleus
(CN IX) and vagus (CN X) efferents- somatic muscles of the pharynx, larynx, and soft palate. (lesion in Wallenberg = hoarse)
-parasympathetic cardiac inhibition - CN X
301
nucleus tractus solitarius - no motor
[Scared in solitary confinement]
Rostral = gustatory-Receive taste inputs VII -chorda typani+ IX - (SVA)
Caudal= IX-carotid body+sinus (9 higher than 10)
X-sensory info from organs, vocal folds, aortic arch, aortic body
-baroreceptor reflex- aortic arch and carotid body
Modulate response to hemostatic changes
Participates in limbic system activity
Participates in carotid reflex, gag reflex, cough reflex, and vomiting reflex
chorda typani: taste and preganglionic PNS to submandibular gland
302
hypnic headache treatment AND nummular HA treatment
Hypnic: -caffeine 1st line
-2nd line-lithium, indomethacin
NOT melatonin
**nummular: gabapentin** 1st line (gabapentin as common as coins)
303
pure motor vs pure sensory vs sensorimotor stroke localization
pure motor - post limb IC
pure sensory - thalamus
mixed - thalamocapsular
304
neurofibroma vs schwannoma vs ganglioneuromas vs perineuroma
neurofibroma: Schwann cells+nerve fibers running through
Schwannomas: only benign Schwann cells, NO nerve fibers running through
perineuroma: perineural cells, pseudo onion bulbing
ganglioneuromas: SYMPATHETIC gangion cells
305
medication overuse HA
OTC drugs - 15 days
ergots, triptans, opioids - 10 days
306
CN V (anterior tense) vs CN VII (posterior style)
CN V3: Muscles mastication, mylohyoid, TENSE, ANTERIOR digastic
-tensor veli palatini,
-tensor tympani (protects from loud noises)
-mylohyoid
-anterior belly digastric
-touch to anterior 2/3 tongue
CN VII: facial expression, STylohyoid, STapedius, POSTERIOR digastric, chorda typani
-stylohyoid
-stapedius middle ear (protects from loud noises)
-posterior belly digastric
-Chorda typani - taste + preganglionic PNS innervation to superior (gustatory) salivatory nuc
**pterygopalatine=sphenopalatine ganglion - PNS innervation to nasal mucosa, lacrimal gland
-travels through parotid but does not innervate
stylopharyngeus - CN IX
307
CGRP inhibitors
erenumab - long term safety and efficacy established - LIBERTY 2021 study -if fail 2-4 preventatives
ubrogepant - CYP3A4 inhibitors contraindicated
308
topiramate
levels decrease in pregnancy
-may decrease effectiveness OCPs
-inhibits AMPA, sodium channel blocker, CA blocker
309
Brodmann area primary motor cortex, Broca, Wernicke
4: primary motor
9- prefrontal
44, 45: Broca
22: Wernicke
5, 7 - posterior parietal
310
atherosclerosis vs vasculitis vs fibromuscular dysplasia histology
atherosclerosis: necrotic core = white, foam cells that are lipids, atheroma cap
-VS arteriolosclerosis: hyaline thickening, NOT lipid deposition
vasculitis - fibrinoid necrosis + sclerosis, NO fatty changes
fibromuscular dysplasia - thickened collagen, muscular hyperplasia no fibrosis
311
should abduction >90 degrees
**trapezius - spinal accessory nerve C3, C4 - cervical lymph node dissection**
serratus anterior - long thoracic -C5-6-7
312
sciatic neuropathy
short head+long head biceps femoris weak
short head biceps femoris = common peroneal
long head = tibial division sciatic nerve
